Last Month on Pubmed

This is a series of the latest published research on Alpha-1 Antitrypsin Deficiency. It is updated monthly, based on a PubMed.gov search.

November 2022 Publications

	[Alpha-1 antitrypsin deficiency]. Marando M, Rayroux C, Bergeron A. Rev Med Suisse. 2022 Nov 16;18(804):2169-2174. doi: 10.53738/REVMED.2022.18.804.2169. PMID: 36382978 French.
2.	Alpha-1-antitrypsin in serum exosomes and pericardial fluid exosomes is associated with severity of rheumatic heart disease. Sharma S, Sarkar S, Choudhury C, Singh L, Singh H, Chakraborti A. Mol Cell Biochem. 2022 Nov 1. doi: 10.1007/s11010-022-04595-x. Online ahead of print. PMID: 36318408
3.	The role of alpha-1-antitrypsin in the etiopathogenesis of chronic venous disease: A prospective clinical trial. Limandal HK, Kayğın MA, Özkaya AL, Özkara T, Diler MS, Çüçen HI, Yıldız Z, Ergün S, Dağ Ö. Phlebology. 2022 Nov 22:2683555221141818. doi: 10.1177/02683555221141818. Online ahead of print. PMID: 36413267
4.	Long-term safety of Prolastin®-C, an alpha1-proteinase inhibitor, in Japanese patients with alpha1- antitrypsin deficiency. Seyama K, Suzuki M, Tasaka S, Nukiwa T, Sato T, Konno S, Sorrells S, Chen J, Aragonés ME, Minamino H. Respir Investig. 2022 Nov;60(6):831-839. doi: 10.1016/j.resinv.2022.07.001. Epub 2022 Aug 12. PMID: 35970714 Clinical Trial.
5.	Optimizing the screening of alpha-1 antitrypsin deficiency using serum protein electrophoresis. Wauthier L, Jacques S, Delanghe J, Favresse J. Clin Chem Lab Med. 2022 Nov 24. doi: 10.1515/cclm-2022-0967. Online ahead of print. PMID: 36420543
6.	Disease Status at Diagnosis in Danish Children with α 1 -antitrypsin Deficiency. Winther CL, Nyrann S, Nielsen RG, Duno M, Johansen KB, Helt TW, Brix Christensen V. J Pediatr Gastroenterol Nutr. 2022 Nov 1;75(5):629-634. doi: 10.1097/MPG.0000000000003604. Epub 2022 Sep 6. PMID: 36070551
7.	Imbalance between alpha-1-antitrypsin and interleukin 6 is associated with in-hospital mortality and thrombosis during COVID-19. Philippe A, Puel M, Narjoz C, Gendron N, Durey-Dragon MA, Vedie B, Balduyck M, Chocron R, Hauw-Berlemont C, Sanchez O, Mirault T, Diehl JL, Smadja DM, Loriot MA. Biochimie. 2022 Nov;202:206-211. doi: 10.1016/j.biochi.2022.07.012. Epub 2022 Aug 8. PMID: 35952950 Free PMC article.
8.	Pulmonary Function and Respiratory Diseases in Different Genotypes of Alpha-1 Antitrypsin Deficiency. Hernández Pérez JM, Suárez Sánchez JJ, López Charry CV, Fariña YR, Pérez Pérez JA. Turk Thorac J. 2022 Nov;23(6):376-382. doi: 10.5152/TurkThoracJ.2022.22001. PMID: 35957569 Free PMC article.
9.	Alpha-1 antitrypsin Pi∗Z allele is an independent risk factor for liver transplantation and death in patients with advanced chronic liver disease. Balcar L, Scheiner B, Urheu M, Weinberger P, Paternostro R, Simbrunner B, Hartl L, Jachs M, Bauer D, Semmler G, Willheim C, Pinter M, Ferenci P, Trauner M, Reiberger T, Stättermayer AF, Mandorfer M. JHEP Rep. 2022 Aug 20;4(11):100562. doi: 10.1016/j.jhepr.2022.100562. eCollection 2022 Nov. PMID: 36176936 Free PMC article.
10.	A randomized controlled pilot trial of etanercept and alpha-1 antitrypsin to improve autologous islet raftment.
	Abdel-Karim TR, Hodges JS, Pruett TL, Ramanathan KV, Hering BJ, Dunn TB, Kirchner VA, Beilman GJ, Bellin MD. Pancreatology. 2022 Nov 24:S1424-3903(22)00795-5. doi: 10.1016/j.pan.2022.11.006. Online ahead of print. PMID: 36443174
11.	Liver cancer in severe alpha-1 antitrypsin deficiency: who is at risk? Fromme M, Strnad P. Eur Respir J. 2022 Nov 3;60(5):2200718. doi: 10.1183/13993003.00718-2022. Print 2022 Nov. PMID: 35595316 No abstract available.
12.	Alpha-1 Antitrypsin Inhibits Tumorigenesis and Progression of Colitis-Associated Colon Cancer through Suppression of Inflammatory Neutrophil-Activated Serine Proteases and IGFBP-3 Proteolysis. Cai Q, Kim M, Harada A, Idowu MO, Sundaresan G, Zweit J, Oh Y. Int J Mol Sci. 2022 Nov 8;23(22):13737. doi: 10.3390/ijms232213737. PMID: 36430216
13.	Cancer risk in severe alpha-1 antitrypsin deficiency: the importance of early identification. Hiller AM, Ekström M, Piitulainen E, Lindberg A, Rönmark E, Tanash H. Eur Respir J. 2022 Nov 3;60(5):2200846. doi: 10.1183/13993003.00846-2022. Print 2022 Nov. PMID: 35595322 No abstract available.

October 2022 Publications

1.	Alpha-1 antitrypsin deficiency. Dasí F. Med Clin (Barc). 2022 Oct 14:S0025-7753(22)00458-4. doi: 10.1016/j.medcli.2022.09.001. Online ahead of print. PMID: 36253208 English, Spanish. No abstract available.
2.	Cancer risk in severe alpha-1-antitrypsin deficiency. Hiller AM, Ekström M, Piitulainen E, Lindberg A, Rönmark E, Tanash H. Eur Respir J. 2022 Oct 27;60(4):2103200. doi: 10.1183/13993003.03200-2021. Print 2022 Oct. PMID: 35361631
3.	[Alpha 1-antitrypsin deficiency]. Mornex JF. Rev Mal Respir. 2022 Oct;39(8):698-707. doi: 10.1016/j.rmr.2022.02.062. Epub 2022 Jun 14. PMID: 35715315 Review. French.
4.	Alpha-1 antitrypsin deficiency-associated panniculitis. Franciosi AN, Ralph J, O’Farrell NJ, Buckley C, Gulmann C, O’Kane M, Carroll TP, McElvaney NG. J Am Acad Dermatol. 2022 Oct;87(4):825-832. doi: 10.1016/j.jaad.2021.01.074. Epub 2021 Jan 29. PMID: 33516773 Review.
5.	Frequency of alpha-1 antitrypsin deficiency and unexpected results in COPD patients in Turkey; rare variants are common. Çörtük M, Demirkol B, Arslan MA, İlhan U, Kalkan YE, Turan D, Gül Ş, Çınarka H, Baydili KN, Çetinkaya E. Turk J Med Sci. 2022 Oct;52(5):1478-1485. doi: 10.55730/1300-0144.5486. Epub 2022 Oct 19. PMID: 36422500
6.	Unique and shared systemic biomarkers for emphysema in Alpha-1 Antitrypsin deficiency and chronic obstructive pulmonary disease. Serban KA, Pratte KA, Strange C, Sandhaus RA, Turner AM, Beiko T, Spittle DA, Maier L, Hamzeh N, Silverman EK, Hobbs BD, Hersh CP, DeMeo DL, Cho MH, Bowler RP. EBioMedicine. 2022 Oct;84:104262. doi: 10.1016/j.ebiom.2022.104262. Epub 2022 Sep 22. PMID: 36155958 Free PMC article.
7.	Patient-Reported Pulmonary Symptoms, Exacerbations, and Management in a Cohort of Patients With Alpha-1 Antitrypsin Deficiency. Choate R, Sandhaus RA, Holm KE, Mannino DM, Strange C. Chronic Obstr Pulm Dis. 2022 Oct 26;9(4):549-561. doi: 10.15326/jcopdf.2022.0317. PMID: 36103189
8.	Cancer risk associated with alpha-1-antitrypsin deficiency: only large registries will give us the answer. Barrecheguren M, Miravitlles M. Eur Respir J. 2022 Oct 27;60(4):2201289. doi: 10.1183/13993003.01289-2022. Print 2022 Oct. PMID: 36302522 No abstract available.
9.	Novel SERPINA1 Alleles Identified through a Large Alpha-1 Antitrypsin Deficiency Screening Program and Review of Known Variants. Wiesemann GS, Oshins RA, Flagg TO, Brantly ML. Chronic Obstr Pulm Dis. 2022 Oct 24. doi: 10.15326/jcopdf.2022.0321. Online ahead of print. PMID: 36367950
10.	A stacking ensemble machine learning model to predict alpha-1 antitrypsin deficiency-associated liver disease clinical outcomes based on UK Biobank data. Meng L, Treem W, Heap GA, Chen J. Sci Rep. 2022 Oct 11;12(1):17001. doi: 10.1038/s41598-022-21389-9. 20873 Free PMC article.
11.	Alpha-1 Antitrypsin Deficiency Severe and No Severe. Is It Benefit or Risk? Hernández Pérez JM, López Charry CV. Arch Bronconeumol. 2022 Oct;58(10):731-732. doi: 10.1016/j.arbres.2022.05.012. Epub 2022 Jun 15. PMID: 35764466 English, Spanish. No abstract available.
12.	Association of the C-terminal 42-peptide fragment of alpha-1 antitrypsin with the severity of ARDS: A pilot study. Schnabel C, Harnisch LO, Walter D, Blaurock-Möller N, Bauer M, Quintel M, Kiehntopf M. Clin Biochem. 2022 Oct 13:S0009-9120(22)00229-6. doi: 10.1016/j.clinbiochem.2022.10.005. Online ahead of print. PMID: 36244468

September 2022 Publications

Alpha-1 antitrypsin in autoimmune diseases: Roles and therapeutic prospects.
Sun R, Xu Z, Zhu C, Chen T, Muñoz LE, Dai L, Zhao Y. Int Immunopharmacol. 2022 Sep;110:109001. doi: 10.1016/j.intimp.2022.109001. Epub 2022 Jul 6.
PMID: 35803133 Review.

Alpha-1 antitrypsin: A novel biomarker and potential therapeutic approach for metabolic diseases.
Zhang X, Ostrov DA, Tian H. Clin Chim Acta. 2022 Sep 1;534:71-76. doi: 10.1016/j.cca.2022.07.005. Epub 2022 Jul 8.
PMID: 35810800 Review.

High-level production of wild-type and oxidation-resistant recombinant alpha-1-antitrypsin in glycoengineered CHO cells.
Koyuturk I, Kedia S, Robotham A, Star A, Brochu D, Sauvageau J, Kelly J, Gilbert M, Durocher Y. Biotechnol Bioeng. 2022 Sep;119(9):2331-2344. doi: 10.1002/bit.28129. Epub 2022 May 25.
PMID: 35508753

Pharmacokinetics and Biochemical Efficacy of an α1-Proteinase Inhibitor (Aralast NP) in α1- Antitrypsin Deficiency: a Cross-Product Retrospective Comparability Analysis.
Li Z, Franke RM, Morris DN, Yel L. Pulm Ther. 2022 Sep;8(3):311-326. doi: 10.1007/s41030-022-00199-4. Epub 2022 Aug 24.
PMID: 36001294 Free PMC article.

Nanomedicine platform for targeting activated neutrophils and neutrophil-platelet complexes using an α1-antitrypsin-derived peptide motif.
Cruz MA, Bohinc D, Andraska EA, Alvikas J, Raghunathan S, Masters NA, van Kleef ND, Bane KL, Hart K, Medrow K, Sun M, Liu H, Haldeman S, Banerjee A, Lessieur EM, Hageman K, Gandhi A, de la Fuente M, Nieman MT, Kern TS, Maas C, de Maat S, Neeves KB, Neal MD, Sen Gupta A, Stavrou EX. Nat Nanotechnol. 2022 Sep;17(9):1004-1014. doi: 10.1038/s41565-022-01161-w. Epub 2022 Jul 18.
PMID: 35851383

Ferret Systemic Coronavirus in Alpha-1 Antitrypsin Knockout Ferrets.
Osborne AJ, Hussain SS, Helman EE, Foote JB, Kiupel M, Rowe SM, Collins DE. Comp Med. 2022 Sep 14. doi: 10.30802/AALAS-CM-22-000035. Online ahead of print.
PMID: 36104147

Correlation of alpha-1 antitrypsin levels and exosome associated neutrophil elastase endothelial injury in subjects with SARS-CoV2 infection.
Lascano J, Oshins R, Eagan C, Wadood Z, Qiang X, Flagg T, Scindia Y, Mehrad B, Brantly M, Khodayari N. PLoS One. 2022 Sep 9;17(9):e0274427. doi: 10.1371/journal.pone.0274427. eCollection 2022.
PMID: 36084115 Free PMC article.

Unique and shared systemic biomarkers for emphysema in Alpha-1 Antitrypsin deficiency and chronic obstructive pulmonary disease.
Serban KA, Pratte KA, Strange C, Sandhaus RA, Turner AM, Beiko T, Spittle DA, Maier L, Hamzeh N, Silverman EK, Hobbs BD, Hersh CP, DeMeo DL, Cho MH, Bowler RP. EBioMedicine. 2022 Sep 22;84:104262. doi: 10.1016/j.ebiom.2022.104262. Online ahead of print.
PMID: 36155958 Free PMC article.

Selective decrease in alpha1-antitrypsin levels in diabetic retinopathy: Could the levels of it be playing a role in the pathophysiology of diabetic retinopathy?
Kunder M, Lakshmaiah V, Moideen Kutty AV. Indian J Med Res. 2022 Sep 20. doi: 10.4103/ijmr.IJMR_1293_19. Online ahead of print.
PMID: 36124492

Cigarette smoke exposed airway epithelial cell-derived EVs promote pro-inflammatory macrophage activation in alpha-1 antitrypsin deficiency.
Khodayari N, Oshins R, Mehrad B, Lascano JE, Qiang X, West JR, Holliday LS, Lee J, Wiesemann G, Eydgahi S, Brantly M.
Respir Res. 2022 Sep 6;23(1):232. doi: 10.1186/s12931-022-02161-z.
PMID: 36068572 Free PMC article.

Alpha-1 antitrypsin deficiency, asthma and pregnancy. Is there a place for augmentative therapy?
Hernández Pérez JM, López Charry CV. Pulmonology. 2022 Sep-Oct;28(5):409-410. doi: 10.1016/j.pulmoe.2022.03.002. Epub 2022 Mar 26.
PMID: 35351399 No abstract available.

Molecular characterization of PI ^* S hangzhou , a SERPINA1 allele from continental China encoding a defective alpha-1-antitrypsin.
Hernández-Pérez JM, González Carracedo MA, García AC, Pérez JAP. Front Pediatr. 2022 Sep 14;10:985892. doi: 10.3389/fped.2022.985892. eCollection 2022.
PMID: 36186645 Free PMC article.

The unfolded protein response to PI*Z alpha-1 antitrypsin in human hepatocellular and murine models.
Lu Y, Wang LR, Lee J, Mohammad NS, Aranyos AM, Gould C, Khodayari N, Oshins RA, Moneypenny CG, Brantly ML.
Hepatol Commun. 2022 Sep;6(9):2354-2367. doi: 10.1002/hep4.1997. Epub 2022 May 27.
PMID: 35621045 Free PMC article.

Patient-Reported Pulmonary Symptoms, Exacerbations, and Management in a Cohort of Patients With Alpha-1 Antitrypsin Deficiency.
Choate R, Sandhaus RA, Holm KE, Mannino DM, Strange C. Chronic Obstr Pulm Dis. 2022 Sep 14. doi: 10.15326/jcopdf.2022.0317. Online ahead of print.
PMID: 36103189

Impact of the PEG length and PEGylation site on the structural, thermodynamic, thermal, and proteolytic stability of mono-PEGylated alpha-1 antitrypsin.
Liu X, Kouassi KGW, Vanbever R, Dumoulin M. Protein Sci. 2022 Sep;31(9):e4392. doi: 10.1002/pro.4392.
PMID: 36040264

Demographic and clinical characteristics of patients with α1-antitrypsin deficiency genotypes PI*ZZ and PI*SZ in the Spanish registry of EARCO.
Torres-Durán M, López-Campos JL, Rodríguez-Hermosa JL, Esquinas C, Martínez-González C, Hernández-Pérez JM, Rodríguez C, Bustamante A, Casas-Maldonado F, Barrecheguren M, González C, Miravitlles M. ERJ Open Res. 2022 Sep 26;8(3):00213-2022. doi: 10.1183/23120541.00213-2022. eCollection 2022 Jul.
PMID: 36171983 Free PMC article.

Augmentation therapy with human alpha-1-proteinase inhibitor reduces exacerbations in patient with bronchiectasis and alpha-1-antitrypsin deficiency.
Buck E, Presotto MA, Brock J, Schlamp K, Veith M, Herth FJF, Trudzinski FC. Respir Med Case Rep. 2022 Sep 14;39:101740. doi: 10.1016/j.rmcr.2022.101740. eCollection 2022. PMID: 36161236 Free PMC article.

Pregnancy in patients with Alpha 1 Antitrypsin (AAT) deficiency and the role of intravenous AAT therapy. Authors’ reply.
Guarnieri G, Achille A, Lococo S, Vianello A.
Pulmonology. 2022 Sep-Oct;28(5):411-412. doi: 10.1016/j.pulmoe.2022.03.009. Epub 2022 Apr 29.
PMID: 35501281 No abstract available.

Alpha-1-antitrypsin deficiency caused by a PI*ZQ0Leiria genotype- the importance of diagnosis algorithms.
Silva MJV, Seixas S, Canotilho MJ, Feijó S.
Pulmonology. 2022 Sep 27:S2531-0437(22)00196-9. doi: 10.1016/j.pulmoe.2022.07.012. Online ahead of print.
PMID: 36180350 No abstract available.

Usefulness of the “La palma” diagnostic algorithm in the alpha-1 antitrypsin deficiency.
Hernández Pérez JM, López Charry CV.
Med Clin (Barc). 2022 Sep 9;159(5):251-252. doi: 10.1016/j.medcli.2022.03.027. Epub 2022 Jun 24.
PMID: 35760607 English, Spanish. No abstract available.

[French clinical practice guidelines for the diagnosis and management of lung disease with alpha 1- antitrypsin deficiency].
Mornex JF, Balduyck M, Bouchecareilh M, Cuvelier A, Epaud R, Kerjouan M, Le Rouzic O, Pison C, Plantier L, Pujazon MC, Reynaud-Gaubert M, Toutain A, Trumbic B, Willemin MC, Zysman M, Brun O, Campana M, Chabot F, Chamouard V, Dechomet M, Fauve J, Girerd B, Gnakamene C, Lefrançois S, Lombard JN, Maitre B, Maynié-François C, Moerman A, Payancé A, Reix P, Revel D, Revel MP, Schuers M, Terrioux P, Theron D, Willersinn F, Cottin V, Mal H.
Rev Mal Respir. 2022 Sep;39(7):633-656. doi: 10.1016/j.rmr.2022.06.002. Epub 2022 Jul 27.
PMID: 35906149 French. No abstract available.

August 2022 Publications

Fazirsiran for Liver Disease Associated with Alpha1-Antitrypsin Deficiency.
Strnad P, Mandorfer M, Choudhury G, Griffiths W, Trautwein C, Loomba R, Schluep T, Chang T, Yi M, Given BD, Hamilton JC, San Martin J, Teckman JH. N Engl J Med. 2022 Aug 11;387(6):514-524. doi: 10.1056/NEJMoa2205416. Epub 2022 Jun 25.
PMID: 35748699 Clinical Trial.

Alpha-1 Antitrypsin Deficiency Liver Disease.
Suri A, Patel D, Teckman JH. Clin Liver Dis. 2022 Aug;26(3):391-402. doi: 10.1016/j.cld.2022.03.004. Epub 2022 Jun 25.
PMID: 35868681 Review.

Long-term safety of Prolastin®-C, an alpha1-proteinase inhibitor, in Japanese patients with alpha1- antitrypsin deficiency.
Seyama K, Suzuki M, Tasaka S, Nukiwa T, Sato T, Konno S, Sorrells S, Chen J, Aragonés ME, Minamino H. Respir Investig. 2022 Aug 12:S2212-5345(22)00096-X. doi: 10.1016/j.resinv.2022.07.001. Online ahead of print.
PMID: 35970714

Imbalance between alpha-1-antitrypsin and interleukin 6 is associated with in-hospital mortality and thrombosis during COVID-19.
Philippe A, Puel M, Narjoz C, Gendron N, Durey-Dragon MA, Vedie B, Balduyck M, Chocron R, Hauw-Berlemont C, Sanchez O, Mirault T, Diehl JL, Smadja DM, Loriot MA. Biochimie. 2022 Aug 8:S0300-9084(22)00188-2. doi: 10.1016/j.biochi.2022.07.012. Online ahead of print.
PMID: 35952950 Free PMC article.

Disease progression in patients with PI*ZZ alpha-1 antitrypsin deficiency.
Shen K, Lyu R, Sengupta S, Wakim-Fleming J. Eur J Gastroenterol Hepatol. 2022 Aug 1;34(8):852-856. doi: 10.1097/MEG.0000000000002395. Epub 2022 Jun 27.
PMID: 35802526

Pulmonary Function and Respiratory Diseases in Different Genotypes of Alpha-1 Antitrypsin Deficiency.
Hernández Pérez JM, Suárez Sánchez JJ, López Charry CV, Fariña YR, Pérez Pérez JA. Turk Thorac J. 2022 Aug 12. doi: 10.5152/TurkThoracJ.2022.22001. Online ahead of print.
PMID: 35957569

Leave no one behind: inclusion of alpha-1 antitrypsin deficiency patients in COVID-19 vaccine trials.
Yang C, Zhao H, Tebbutt SJ. Eur J Hum Genet. 2022 Aug;30(8):872-874. doi: 10.1038/s41431-022-01047-4. Epub 2022 Jan 28.
PMID: 35087186 Free PMC article.

Alpha-1 antitrypsin Pi∗Z allele is an independent risk factor for liver transplantation and death in patients with advanced chronic liver disease.
Balcar L, Scheiner B, Urheu M, Weinberger P, Paternostro R, Simbrunner B, Hartl L, Jachs M, Bauer D, Semmler G, Willheim C, Pinter M, Ferenci P, Trauner M, Reiberger T, Stättermayer AF, Mandorfer M. JHEP Rep. 2022 Aug 20;4(11):100562. doi: 10.1016/j.jhepr.2022.100562. eCollection 2022 Nov.
PMID: 36176936 Free PMC article.

The investigation of the frequency of the alpha-1-antitrypsin phenotype in patients with liver cirrhosis.
Afsharinasab M, Akbari AH, Mirzaei V, Mahmoodi M, Hajizadeh MR, Amri J, Khoshdel A. Horm Mol Biol Clin Investig. 2022 Aug 17. doi: 10.1515/hmbci-2022-0001. Online ahead of print.
PMID: 35973212

Lower serum alpha 1 antitrypsin levels in patients with severe COVID-19 compared with patients hospitalized due to non-COVID-19 pneumonia.
Pertzov B, Shapira G, Abushkara S, Cohen S, Turjeman A, Kramer MR, Gurwitz D, Shomron N. Infect Dis (Lond). 2022 Aug 17:1-6. doi: 10.1080/23744235.2022.2111464. Online ahead of print.
PMID: 35975662

Occupational and environmental exposures in the Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis (GRADS) study.
Ryan SM, Mroz MM, Herzog EL, Ryu C, Fingerlin TE, Maier LA, Gulati M. Respir Med. 2022 Aug-Sep;200:106923. doi: 10.1016/j.rmed.2022.106923. Epub 2022 Jun 30.
PMID: 35932543

Development of peptide ligands for the purification of α-1 antitrypsin from cell culture fluids.
Chu W, Prodromou R, Moore B, Elhanafi D, Kilgore R, Shastry S, Menegatti S. J Chromatogr A. 2022 Aug 30;1679:463363. doi: 10.1016/j.chroma.2022.463363. Epub 2022 Jul 22.
PMID: 35964464

July 2022 Publications

Alpha 1 Antitrypsin Deficiency.
Meseeha M, Attia M. 2022 Jul 19. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan–.
PMID: 28723059 Free Books & Documents.

Alpha 1 Antitrypsin Mutation.
Abdulkarim A, Craig TJ. 2022 Jul 12. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan–.
PMID: 29489299 Free Books & Documents.

Alpha-1 antitrypsin deficiency hidden in allegedly normal variants.
Suárez-Lorenzo I, Hernández-Brito E, Almeida-Quintana L, Llanos CG, González-Quevedo N, Carrillo-Díaz T, Rodríguez-Gallego C. J Asthma. 2022 Jul;59(7):1372-1375. doi: 10.1080/02770903.2021.1944186. Epub 2021 Jul 13.
PMID: 34152895

Expert Perspectives on the Management of Alpha 1-Antitrypsin Deficiency.
Conde B, Costa F, Gomes J, Lopes AP, Mineiro MA, Rodrigues O, Santos C, Semedo L, Sucena M, Guimarães C. Acta Med Port. 2022 Jul 18. doi: 10.20344/amp.18487. Online ahead of print.
PMID: 35848753 Review.

Alpha-1 Antitrypsin Deficiency: Does Increased Neutrophil Adhesion Contribute to Lung Damage?
Prendecki M, Lodge KM. Am J Respir Cell Mol Biol. 2022 Jul;67(1):6-7. doi: 10.1165/rcmb.2022-0100ED.
PMID: 35522757 Free PMC article. No abstract available.

Alpha-1-Antitrypsin Experience for Steroid-Resistant Acute Graft-Versus-Host Disease.
Koyun D, Seval GC, Kırcalı E, Bozdağ SC, Toprak SK, Topçuoğlu P, Özcan M, Arslan Ö, Demirer T, İlhan O, Beksaç M, Gürman G, Yüksel MK. Indian J Hematol Blood Transfus. 2022 Jul;38(3):601-605. doi: 10.1007/s12288-022-01524-2. Epub 2022 Feb 7.
PMID: 35747565

Alpha-1 Antitrypsin Therapy Modifies Neutrophil Adhesion in Patients with Obstructive Lung Disease.
McEnery T, White MM, Gogoi D, Coleman O, Bergin D, Jundi B, Flannery R, Alsaif FAT, Landers SA, Casey M, Dunlea D, Meleady P, McElvaney NG, Reeves EP.
Am J Respir Cell Mol Biol. 2022 Jul;67(1):76-88. doi: 10.1165/rcmb.2021-0433OC.
PMID: 35507773

Assessing Patients with Alpha-1 Antitrypsin Deficiency for Corneal Refractive Surgery: A Review and Clinical Experience.
Moshirfar M, Kelkar N, Ronquillo YC, Hoopes PC. J Clin Med. 2022 Jul 19;11(14):4175. doi: 10.3390/jcm11144175.
PMID: 35887939 Free PMC article.

Alpha-1 Antitrypsin Reduces Disease Progression in a Mouse Model of Charcot-Marie-Tooth Type 1A: A Role for Decreased Inflammation and ADAM-17 Inhibition.
Zhukovsky N, Silvano M, Filloux T, Gonzalez S, Krause KH. Int J Mol Sci. 2022 Jul 3;23(13):7405. doi: 10.3390/ijms23137405.
PMID: 35806409 Free PMC article.

Intravenous Alpha-1 Antitrypsin Therapy for Critically Ill COVID-19 Patients.
Wand O, Dahan D, Tov N, Epstein Shochet G, King DA, Shitrit D. Isr Med Assoc J. 2022 Jul;24(7):441-443.
PMID: 35819210 No abstract available.

Demographic and clinical characteristics of patients with α1-antitrypsin deficiency genotypes PI*ZZ and PI*SZ in the Spanish registry of EARCO.
Torres-Durán M, López-Campos JL, Rodríguez-Hermosa JL, Esquinas C, Martínez-González C, Hernández-Pérez JM, Rodríguez C, Bustamante A, Casas-Maldonado F, Barrecheguren M, González C, Miravitlles M.
ERJ Open Res. 2022 Sep 26;8(3):00213-2022. doi: 10.1183/23120541.00213-2022. eCollection 2022 Jul.
PMID: 36171983 Free PMC article.

Cecropia pachystachya Trécul: identification, isolation of secondary metabolites, in silico study of toxicological evaluation and interaction with the enzymes 5-LOX and α-1-antitrypsin.
Sousa Mourão P, de Oliveira Gomes R, Crisóstomo Bezerra Costa CA, da Silva Moura OF, Sousa HG, Lemos Martins Júnior GR, Cabral Leão Ferreira D, Martins Maia Filho AL, Duarte de Freitas J, Rai M, Das Chagas Alves Lima F, Gourlart Santana AE, Chaves MH, Dos Santos Alves W, Uchôa VT.
J Toxicol Environ Health A. 2022 Oct 18;85(20):827-849. doi: 10.1080/15287394.2022.2095546.
Epub 2022 Jul 11. PMID: 35815836

Pregnancy in Alpha 1 Antitrypsin (AAT) Deficiency and the role of intravenous AAT therapy.
Guarnieri G, Achille A, Lococo S, Vianello A.
Pulmonology. 2022 Jul-Aug;28(4):317-319. doi: 10.1016/j.pulmoe.2022.01.014. Epub 2022 Feb 24.
PMID: 35221261 No abstract available.

Fragments of alpha-1-antitrypsin in patients with severe COVID-19 and bacterial pulmonary sepsis.
Bigalke A, Neu C, Esper Treml R, Coldewey SM, Kiehntopf M. Clin Chem Lab Med. 2022 May 4;60(8):e187-e189. doi: 10.1515/cclm-2022-0361. Print 2022 Jul 26.
PMID: 35503554 No abstract available.

[Impact of COVID-19 pandemic on information management and adherence to replacement therapy with AAT of patients with alpha-1 antitrypsin deficiency (AATM)].
Köhnlein T, Wilkens M, Eydt K. Pneumologie. 2022 Jul;76(7):494-498. doi: 10.1055/a-1845-0160. Epub 2022 Jun 20.
PMID: 35724679 German.

[French clinical practice guidelines for the diagnosis and management of lung disease with alpha 1- antitrypsin deficiency].
Mornex JF, Balduyck M, Bouchecareilh M, Cuvelier A, Epaud R, Kerjouan M, Le Rouzic O, Pison C, Plantier L, Pujazon MC, Reynaud-Gaubert M, Toutain A, Trumbic B, Willemin MC, Zysman M, Brun O, Campana M, Chabot F, Chamouard V, Dechomet M, Fauve J, Girerd B, Gnakamene C, Lefrançois S, Lombard JN, Maitre B, Maynié-François C, Moerman A, Payancé A, Reix P, Revel D, Revel MP, Schuers M, Terrioux P, Theron D, Willersinn F, Cottin V, Mal H. Rev Mal Respir. 2022 Sep;39(7):633-656. doi: 10.1016/j.rmr.2022.06.002. Epub 2022 Jul 27.
PMID: 35906149 French. No abstract available.

IMpact of COVID-19 in Patients With Severe Alpha-1 Antitrypsin Deficiency: The IMCA1 Study of the EARCO Clinical Research Collaboration.
Parr DG, Chorostowska-Wynimko J, Corsico A, Esquinas C, McElvaney GN, Sark AD, Sucena M, Tanash H, Turner AM, Miravitlles M. Arch Bronconeumol. 2022 Jul 11:S0300-2896(22)00488-4. doi: 10.1016/j.arbres.2022.07.002. Online ahead of print.
PMID: 35879122 Free PMC article. No abstract available.

June 2022 Publications

Fazirsiran for Liver Disease Associated with Alpha1-Antitrypsin Deficiency.
Strnad P, Mandorfer M, Choudhury G, Griffiths W, Trautwein C, Loomba R, Schluep T, Chang T, Yi M, Given BD, Hamilton JC, San Martin J, Teckman JH.
N Engl J Med. 2022 Aug 11;387(6):514-524. doi: 10.1056/NEJMoa2205416. Epub 2022 Jun 25.
PMID: 35748699 Clinical Trial.

[Alpha 1-antitrypsin deficiency].
Mornex JF. Rev Mal Respir. 2022 Jun 14:S0761-8425(22)00159-0. doi: 10.1016/j.rmr.2022.02.062. Online ahead of print.
PMID: 35715315 Review. French.

Alpha-1 Antitrypsin Deficiency Liver Disease.
Suri A, Patel D, Teckman JH.
Clin Liver Dis. 2022 Aug;26(3):391-402. doi: 10.1016/j.cld.2022.03.004. Epub 2022 Jun 25.
PMID: 35868681 Review.

Low Prevalence of Mild Alpha-1-Antitrypsin Deficiency in Hospitalized COVID-19-Patients.
Nygren D, Mölstad U, Thulesius H, Hillman M, Broman LM, Tanash H, Landin-Olsson M, Rasmussen M, Thunander M. Int J Gen Med. 2022 Jun 28;15:5843-5848. doi: 10.2147/IJGM.S370434. eCollection 2022.
PMID: 35789772 Free PMC article.

Comparative biochemical efficacy analysis of an alpha1-proteinase inhibitor (Glassia®) in patients with alpha-1 antitrypsin deficiency.
Li Z, Franke RM, Morris D, Yel L. Pulm Pharmacol Ther. 2022 Jun;73-74:102124. doi: 10.1016/j.pupt.2022.102124. Epub 2022 Mar 18.
PMID: 35314325

Alpha-1 Antitrypsin Deficiency Severe and No Severe. Is It Benefit or Risk?
Hernández Pérez JM, López Charry CV. Arch Bronconeumol. 2022 Jun 15:S0300-2896(22)00444-6. doi: 10.1016/j.arbres.2022.05.012. Online ahead of print.
PMID: 35764466 English, Spanish. No abstract available.

Hepatic decompensation is accelerated in patients with cirrhosis and alpha-1 antitrypsin Pi∗MZ genotype.
Chen VL, Burkholder DA, Moran IJ, DiBattista JV, Miller MJ, Chen Y, Du X, Oliveri A, Cushing KC, Lok AS, Speliotes EK.
JHEP Rep. 2022 Apr 5;4(6):100483. doi: 10.1016/j.jhepr.2022.100483. eCollection 2022 Jun.
PMID: 35571533 Free PMC article.

Serum alpha-1-antitrypsin level in the severity prognosis of systemic lupus erythematosus patients: Systematic exploration of novel biomarker.
Khamchun S, Thakaeng C, Na Lampang R. Biomedicine (Taipei). 2022 Jun 1;12(2):19-30. doi: 10.37796/2211-8039.1297. eCollection 2022.
PMID: 35836976 Free PMC article.

Alu RNA induces NLRP3 expression through TLR7 activation in α-1-antitrypsin-deficient macrophages.
Lee J, Mohammad N, Lu Y, Kang K, Han K, Brantly M. JCI Insight. 2022 Jun 22;7(12):e158791. doi: 10.1172/jci.insight.158791.
PMID: 35730566 Free PMC article.

Occupational and environmental exposures in the Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis (GRADS) study.
Ryan SM, Mroz MM, Herzog EL, Ryu C, Fingerlin TE, Maier LA, Gulati M.
Respir Med. 2022 Aug-Sep;200:106923. doi: 10.1016/j.rmed.2022.106923. Epub 2022 Jun 30.
PMID: 35932543

Changes in the Expression of Insulin Pathway, Neutrophil Elastase and Alpha 1 Antitrypsin Genes from Leukocytes of Young Individuals with Insulin Resistance.
Cruz-Pineda WD, Garibay-Cerdenares OL, Rodríguez-Ruíz HA, Matia-García I, Marino-Ortega LA, Espinoza-Rojo M, Reyes-Castillo Z, Castro-Alarcón N, Castañeda-Saucedo E, Illades-Aguiar B, Parra-Rojas I. Diabetes Metab Syndr Obes. 2022 Jun 18;15:1865-1876. doi: 10.2147/DMSO.S362881. eCollection 2022.
PMID: 35757193 Free PMC article.

Assessment of the circulatory concentrations of cathepsin D, cathepsin K, and alpha-1 antitrypsin in patients with knee osteoarthritis.
Khoshdel A, Forootan M, Afsharinasab M, Rezaian M, Abbasifard M.
Ir J Med Sci. 2022 Jun 24. doi: 10.1007/s11845-022-03061-3. Online ahead of print.
PMID: 35749030

Cytokine Regulation by Alpha-1 Antitrypsin Therapy: A Pathway Analysis of a Pilot Clinical Trial.
Campos MA, Geraghty P. Am J Respir Cell Mol Biol. 2022 Jun;66(6):697-700. doi: 10.1165/rcmb.2021-0503LE.
PMID: 35648091 No abstract available.

The Null Q0Ourém Variant within a Copy-Neutral Loss-of-Heterozygosity Event Causing Alpha-1 Antitrypsin Deficiency.
Gaudreault N, Blouin C, Haillot A, Milot J, Maltais F, Bossé Y. Am J Respir Cell Mol Biol. 2022 Jun;66(6):700-702. doi: 10.1165/rcmb.2021-0564LE.
PMID: 35648089 No abstract available.

Usefulness of the “La palma” diagnostic algorithm in the alpha-1 antitrypsin deficiency.
Hernández Pérez JM, López Charry CV. Med Clin (Barc). 2022 Sep 9;159(5):251-252. doi: 10.1016/j.medcli.2022.03.027. Epub 2022 Jun 24.
PMID: 35760607 English, Spanish. No abstract available.

May 2022 Publications

Alpha 1 Antitrypsin Deficiency.
Meseeha M, Attia M.
2022 May 10. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan–.
PMID: 28723059 Free Books & Documents.

High-level production of wild-type and oxidation-resistant recombinant alpha-1-antitrypsin in glycoengineered CHO cells.
Koyuturk I, Kedia S, Robotham A, Star A, Brochu D, Sauvageau J, Kelly J, Gilbert M, Durocher Y. Biotechnol Bioeng. 2022 May 4. doi: 10.1002/bit.28129. Online ahead of print.
PMID: 35508753

Alpha-1 Antitrypsin Therapy Modifies Neutrophil Adhesion in Patients with Obstructive Lung Disease.
McEnery T, White MM, Gogoi D, Coleman O, Bergin D, Jundi B, Flannery R, Alsaif FAT, Landers SA, Casey M, Dunlea D, Meleady P, McElvaney NG, Reeves EP.
Am J Respir Cell Mol Biol. 2022 May 4. doi: 10.1165/rcmb.2021-0433OC. Online ahead of print. PMID: 35507773

Liver cancer in severe alpha-1 antitrypsin deficiency: Who is at risk?
Fromme M, Strnad P.
Eur Respir J. 2022 May 20:2200718. doi: 10.1183/13993003.00718-2022. Online ahead of print. PMID: 35595316 No abstract available.

The unfolded protein response to PI*Z alpha-1 antitrypsin in human hepatocellular and murine models.
Lu Y, Wang LR, Lee J, Mohammad NS, Aranyos AM, Gould C, Khodayari N, Oshins RA, Moneypenny CG, Brantly ML.
Hepatol Commun. 2022 May 27. doi: 10.1002/hep4.1997. Online ahead of print. PMID: 35621045

Cancer risk in severe alpha-1-antitrypsin deficiency – the importance of the early identification. Hiller AM, Ekström M, Piitulainen E, Lindberg A, Rönmark E, Tanash H.
Eur Respir J. 2022 May 20:2200846. doi: 10.1183/13993003.00846-2022. Online ahead of print. PMID: 35595322 No abstract available.

Fragments of alpha-1-antitrypsin in patients with severe COVID-19 and bacterial pulmonary sepsis.
Bigalke A, Neu C, Esper Treml R, Coldewey SM, Kiehntopf M.
Clin Chem Lab Med. 2022 May 4. doi: 10.1515/cclm-2022-0361. Online ahead of print. PMID: 35503554 No abstract available.

Alpha1 Antitrypsin Deficiency: Does Increased Neutrophil Adhesion Contribute to Lung Damage?
Prendecki M, Lodge KM.
Am J Respir Cell Mol Biol. 2022 May 6. doi: 10.1165/rcmb.2022-0100ED. Online ahead of print. PMID: 35522757 No abstract available.

Is the determination of C-reactive protein really necessary in the diagnosis of Alpha-1 antitrypsin deficiency?
Hernández-Pérez JM, López-Charry CV.
Clin Respir J. 2022 May;16(5):420-422. doi: 10.1111/crj.13494. Epub 2022 May 17. PMID: 35578817 No abstract available.

Clinical manifestations of a new alpha-1 antitrypsin genetic variant: Q0parma.
Aiello M, Frizzelli A, Marchi L, Ferrarotti I, Piloni D, Pelà G, De Simoni A, D’Aloisio L, Calzetta L, Chetta A.
Respirol Case Rep. 2022 Apr 14;10(5):e0936. doi: 10.1002/rcr2.936. eCollection 2022 May. PMID: 35433011 Free PMC article.

Developing lung cancer in COPD: Possible role of carrying Alpha-1 antitrypsin deficiency variants.
Onur ST, Boyracı N, Akyıl FT, Sökücü SN, Kara K.
Respir Med Case Rep. 2022 May 21;38:101667. doi: 10.1016/j.rmcr.2022.101667. eCollection
2022.
PMID: 35646586 Free PMC article.

Term pregnancy of a patient with severe pulmonary emphysema associated with (PI*ZZ) alpha-1 antitrypsin.
Martínez-González C, Adanez Garcia J, Blanco I.
Arch Bronconeumol. 2022 May;58(5):427-428. doi: 10.1016/j.arbres.2022.02.004. Epub 2022 Feb 12.
PMID: 35312536 English, Spanish. No abstract available.

Cystic fibrosis and alpha-1 antitrypsin deficiency: case report and review of literature.
Jaspers E, Van Dijck I, Hoffman I, Knops N, Stéphenne X, Witters P, Proesmans M. BMC Pediatr. 2022 May 3;22(1):247. doi: 10.1186/s12887-022-03290-6.
PMID: 35505316 Free PMC article. Review.

[Translated article] Term Pregnancy in a Patient With Severe Pulmonary Emphysema Associated With Pi*ZZ Alpha-1 Antitrypsin Deficiency.
Martínez-González C, Garcia JA, Blanco I.
Arch Bronconeumol. 2022 May;58(5):T427-T428. doi: 10.1016/j.arbres.2022.02.013. Epub 2022 May 6.
PMID: 35527081 English, Spanish. No abstract available.

April 2022 Publications

MZ Alpha-1 Antitrypsin Deficiency.
McElvaney OJ, Finnerty G, Carroll TP, Brantly ML, McElvaney NG.
Am J Respir Crit Care Med. 2022 Apr 1;205(7):P13-P14. doi: 10.1164/rccm.2057P13. PMID: 35361070 No abstract available.

Comparison of alpha 1- antitrypsin activity and phenotype in type 1 diabetic patients to healthy individuals.
Ghoreishi AS, Mahmoodi M, Khoshdel A.
J Family Med Prim Care. 2022 Apr;11(4):1377-1381. doi: 10.4103/jfmpc.jfmpc_905_21. Epub 2022 Mar 18.
PMID: 35516706 Free PMC article.

Alpha-1 antitrypsin deficiency: A re-surfacing adult liver disorder.
Fromme M, Schneider CV, Trautwein C, Brunetti-Pierri N, Strnad P.J Hepatol. 2022 Apr;76(4):946-958. doi: 10.1016/j.jhep.2021.11.022. Epub 2021 Nov 27. PMID: 34848258 Review.

A randomized, double-blind, placebo-controlled trial of intravenous alpha-1 antitrypsin for ARDS secondary to COVID-19.
McElvaney OJ, McEvoy NL, Boland F, McElvaney OF, Hogan G, Donnelly K, Friel O, Browne E, Fraughen DD, Murphy MP, Clarke J, Choileáin ON, O’Connor E, McGuinness R, Boylan M, Kelly A, Hayden JC, Collins AM, Cullen A, Hyland D, Carroll TP, Geoghegan P, Laffey JG, Hennessy M, Martin-Loeches I, McElvaney NG, Curley GF.
Med (N Y). 2022 Apr 8;3(4):233-248.e6. doi: 10.1016/j.medj.2022.03.001. Epub 2022 Mar 11.
PMID: 35291694 Free PMC article. Clinical Trial.

Attitudes Towards Vaccination for Coronavirus Disease 2019 in Patients with Severe Alpha-1 Antitrypsin Deficiency.
McElvaney OJ, Cleary B, Fraughen DD, Kelly G, Murphy MP, McElvaney OF, Branagan P, Gunaratnam C, Carroll TP, McElvaney NG.
Chronic Obstr Pulm Dis. 2022 Apr 29;9(2):266-273. doi: 10.15326/jcopdf.2022.0288.
PMID: 35403416

Z-α1-antitrypsin polymers impose molecular filtration in the endoplasmic reticulum after undergoing phase transition to a solid state.
Chambers JE, Zubkov N, Kubánková M, Nixon-Abell J, Mela I, Abreu S, Schwiening M, Lavarda G, López-Duarte I, Dickens JA, Torres T, Kaminski CF, Holt LJ, Avezov E, Huntington JA, George-Hyslop PS, Kuimova MK, Marciniak SJ.
Sci Adv. 2022 Apr 8;8(14):eabm2094. doi: 10.1126/sciadv.abm2094. Epub 2022 Apr 8. PMID: 35394846 Free PMC article.

How are we in Brazil with the treatment of alpha-1 antitrypsin deficiency?
Castellano MVCO, Feitosa PH.
J Bras Pneumol. 2022 Apr 29;48(3):e20210045. doi: 10.36416/1806-3756/e20210045.
PMID: 35508062 Free PMC article. No abstract available.

Pregnancy in patients with Alpha 1 Antitrypsin (AAT) deficiency and the role of intravenous AAT therapy. Authors’ reply.
Guarnieri G, Achille A, Lococo S, Vianello A.
Pulmonology. 2022 Apr 29:S2531-0437(22)00081-2. doi: 10.1016/j.pulmoe.2022.03.009. Online ahead of print.
PMID: 35501281 No abstract available.

Author Correction: Enoxaparin augments alpha-1-antitrypsin inhibition of TMPRSS2, a promising drug combination against COVID-19.
Bai X, Buckle AM, Vladar EK, Janoff EN, Khare R, Ordway D, Beckham D, Fornis LB, Majluf- Cruz A, Fugit RV, Freed BM, Kim S, Sandhaus RA, Chan ED.
Sci Rep. 2022 Apr 19;12(1):6466. doi: 10.1038/s41598-022-10890-w.
PMID: 35440617 Free PMC article. No abstract available.

Evaluation of cytosine base editing and adenine base editing as a potential treatment for alpha-1 antitrypsin deficiency.
Packer MS, Chowdhary V, Lung G, Cheng LI, Aratyn-Schaus Y, Leboeuf D, Smith S, Shah A, Chen D, Zieger M, Cafferty BJ, Yan B, Ciaramella G, Gregoire FM, Mueller C.
Mol Ther. 2022 Apr 6;30(4):1396-1406. doi: 10.1016/j.ymthe.2022.01.040. Epub 2022 Feb 2.
PMID: 35121111

March 2022 Publications

Alpha-1-Antitrypsin Deficiency. Suri A, Patel D, Teckman J.
Clin Liver Dis (Hoboken). 2022 Mar 27;19(3):89-92. doi: 10.1002/cld.1147. eCollection 2022 Mar.
PMID: 35355837 Free PMC article. Review.

Labelling Alpha-1 antitrypsin deficiency in the medical record – A call to action.
Riley L, Lascano J.
Respir Med. 2022 Mar;193:106749. doi: 10.1016/j.rmed.2022.106749. Epub 2022 Feb 1.
PMID: 35124356

Frequency of alleles and genotypes associated with alpha-1 antitrypsin deficiency in clinical and general populations: Revelations about underdiagnosis.
Hernández-Pérez JM, Ramos-Díaz R, Vaquerizo-Pollino C, Pérez JA.
Pulmonology. 2022 Mar 25:S2531-0437(22)00030-7. doi: 10.1016/j.pulmoe.2022.01.017. Online ahead of print.
PMID: 35346640

Cancer risk in severe alpha-1-antitrypsin deficiency.
Hiller AM, Ekström M, Piitulainen E, Lindberg A, Rönmark E, Tanash H.
Eur Respir J. 2022 Mar 31:2103200. doi: 10.1183/13993003.03200-2021. Online ahead of print. PMID: 35361631

Ferret models of alpha-1 antitrypsin deficiency develop lung and liver disease.
He N, Liu X, Vegter AR, Evans TIA, Gray JS, Guo J, Moll SR, Guo LJ, Luo M, Ma N, Sun X, Liang B, Yan Z, Feng Z, Qi L, Joshi AS, Shahin W, Yi Y, Gibson-Corley KN, Hoffman EA, Wang K, Mueller C, Engelhardt JF, Rosen BH.
JCI Insight. 2022 Mar 8;7(5):e143004. doi: 10.1172/jci.insight.143004.
PMID: 35104244

Comparative biochemical efficacy analysis of an alpha1-proteinase inhibitor (Glassia®) in patients with alpha-1 antitrypsin deficiency.
Li Z, Franke RM, Morris D, Yel L.
Pulm Pharmacol Ther. 2022 Mar 18:102124. doi: 10.1016/j.pupt.2022.102124. Online ahead of print.
PMID: 35314325

Multiplex quantification of C-terminal alpha-1-antitrypsin peptides provides a novel approach for characterizing systemic inflammation.
Bigalke A, Sponholz C, Schnabel C, Bauer M, Kiehntopf M.
Sci Rep. 2022 Mar 9;12(1):3844. doi: 10.1038/s41598-022-07752-w.
PMID: 35264629 Free PMC article.

Disease burden associated with alpha-1 antitrypsin deficiency: systematic and structured literature reviews.
Miravitlles M, Herepath M, Priyendu A, Sharma S, Vilchez T, Vit O, Haensel M, Lepage V, Gens H, Greulich T.
Eur Respir Rev. 2022 Mar 23;31(163):210262. doi: 10.1183/16000617.0262-2021. Print 2022 Mar 31.
PMID: 35321931 Review.

Alpha-1 antitrypsin deficiency, asthma and pregnancy. Is there a place for augmentative therapy?
Hernández Pérez JM, López Charry CV.
Pulmonology. 2022 Mar 26:S2531-0437(22)00074-5. doi: 10.1016/j.pulmoe.2022.03.002. Online ahead of print.
PMID: 35351399 No abstract available.

Enoxaparin augments alpha-1-antitrypsin inhibition of TMPRSS2, a promising drug combination against COVID-19.
Bai X, Buckle AM, Vladar EK, Janoff EN, Khare R, Ordway D, Beckham D, Fornis LB, Majluf- Cruz A, Fugit RV, Freed BM, Kim S, Sandhaus RA, Chan ED.
Sci Rep. 2022 Mar 25;12(1):5207. doi: 10.1038/s41598-022-09133-9.
PMID: 35338216 Free PMC article.

Assessment of Patient Perspectives and Barriers to Self-Infusion of Augmentation Therapy for Alpha-1 Antitrypsin Deficiency During the COVID-19 Pandemic.
Colello J, Ptasinski A, Zhan X, Kaur S, Craig T. Pulm Ther. 2022 Mar;8(1):95-103. doi: 10.1007/s41030-022-00182-z. Epub 2022 Jan 24.
PMID: 35067906 Free PMC article.

Severe α1-antitrypsin deficiency associated with lower blood pressure and reduced risk of ischemic heart disease: a cohort study of 91,540 individuals and a meta-analysis.
Winther SV, Ahmed D, Al-Shuweli S, Landt EM, Nordestgaard BG, Seersholm N, Dahl M. Respir Res. 2022 Mar 9;23(1):55. doi: 10.1186/s12931-022-01973-3.
PMID: 35264159 Free PMC article.

A randomized, double-blind, placebo-controlled trial of intravenous alpha-1 antitrypsin for acute respiratory distress syndrome secondary to COVID-19.
McElvaney OJ, McEvoy NL, Boland F, McElvaney OF, Hogan G, Donnelly K, Friel O, Browne E, Fraughen DD, Murphy MP, Clarke J, Choileáin ON, O’Connor E, McGuinness R, Boylan M, Kelly A, Hayden JC, Collins AM, Cullen A, Hyland D, Carroll TP, Geoghegan P, Laffey JG, Hennessey M, Martin-Loeches I, McElvaney NG, Curley GF.
Med (N Y). 2022 Mar 11. doi: 10.1016/j.medj.2022.03.001. Online ahead of print. PMID: 35291694
Free PMC article.

February 2022 Publications

Alpha-1 antitrypsin deficiency: clarifying the role of the putative protective threshold.
Franciosi AN, Fraughen D, Carroll TP, McElvaney NG.
Eur Respir J. 2022 Feb 10;59(2):2101410. doi: 10.1183/13993003.01410-2021. Print 2022 Feb.

Alpha 1 Antitrypsin Regulates Trophoblast Syncytialization and Inflammatory Factor Expression.
Yoshida K, Yano A, Kusama K, Ishikawa G, Tamura K.
Int J Mol Sci. 2022 Feb 10;23(4):1955. doi: 10.3390/ijms23041955.
PMID: 35216073 Free PMC article.

Hepatobiliary phenotypes of adults with alpha-1 antitrypsin deficiency.
Fromme M, Schneider CV, Pereira V, Hamesch K, Pons M, Reichert MC, Benini F, Ellis P, H Thorhauge K, Mandorfer M, Burbaum B, Woditsch V, Chorostowska-Wynimko J, Verbeek J, Nevens F, Genesca J, Miravitlles M, Nuñez A, Schaefer B, Zoller H, Janciauskiene S, Abreu N, Jasmins L, Gaspar R, Liberal R, Macedo G, Mahadeva R, Gomes C, Schneider KM, Trauner M, Krag A, Gooptu B, Thorburn D, Marshall A, Hurst JR, Lomas DA, Lammert F, Gaisa NT, Clark V, Griffiths W, Trautwein C, Turner AM, McElvaney NG, Strnad P.
Gut. 2022 Feb;71(2):415-423. doi: 10.1136/gutjnl-2020-323729. Epub 2021 Feb 25.
PMID: 33632708

Evaluation of cytosine base editing and adenine base editing as a potential treatment for alpha-1 antitrypsin deficiency.
Packer MS, Chowdhary V, Lung G, Cheng LI, Aratyn-Schaus Y, Leboeuf D, Smith S, Shah A, Chen D, Zieger M, Cafferty BJ, Yan B, Ciaramella G, Gregoire FM, Mueller C.
Mol Ther. 2022 Feb 2:S1525-0016(22)00079-X. doi: 10.1016/j.ymthe.2022.01.040. Online ahead of print.
PMID: 35121111

Ceruletide and Alpha-1 Antitrypsin as a Novel Combination Therapy for Ischemic Stroke.
Simats A, Ramiro L, Valls R, de Ramón H, García-Rodríguez P, Orset C, Artigas L, Sardon T, Rosell A, Montaner J.
Neurotherapeutics. 2022 Feb 28. doi: 10.1007/s13311-022-01203-0. Online ahead of print. PMID: 35226340

Alpha-1 Antitrypsin MZ Heterozygosity Is an Endotype of Chronic Obstructive Pulmonary Disease.
Ghosh AJ, Hobbs BD, Moll M, Saferali A, Boueiz A, Yun JH, Sciurba F, Barwick L, Limper AH, Flaherty K, Criner G, Brown KK, Wise R, Martinez FJ, Lomas D, Castaldi PJ, Carey VJ, DeMeo DL, Cho MH, Silverman EK, Hersh CP; COPDGene Investigators.
Am J Respir Crit Care Med. 2022 Feb 1;205(3):313-323. doi: 10.1164/rccm.202106-1404OC. PMID: 34762809

Proteoform Profiles Reveal That Alpha-1-Antitrypsin in Human Serum and Milk Is Derived From a Common Source.
Jager S, Cramer DAT, Hoek M, Mokiem NJ, van Keulen BJ, van Goudoever JB, Dingess KA, Heck AJR.
Front Mol Biosci. 2022 Feb 22;9:858856. doi: 10.3389/fmolb.2022.858856. eCollection 2022. PMID: 35274008 Free PMC article.

Sensitive and specific measurement of alpha1-antitrypsin activity with an elastase complex formation immunosorbent assay (ECFISA).
Engelmaier A, Weber A.
J Pharm Biomed Anal. 2022 Feb 5;209:114476. doi: 10.1016/j.jpba.2021.114476. Epub 2021 Nov 23.
PMID: 34838346

Recent advancements in understanding the genetic involvement of alpha-1 antitrypsin deficiency associated lung disease: a look at future precision medicine approaches.
Ghosh AJ, Hobbs BD.
Expert Rev Respir Med. 2022 Feb;16(2):173-182. doi: 10.1080/17476348.2022.2027755. Epub 2022 Jan 13.
PMID: 35025710 Free PMC article. Review.

A Review of Alpha-1 Antitrypsin Binding Partners for Immune Regulation and Potential Therapeutic Application.
O’Brien ME, Murray G, Gogoi D, Yusuf A, McCarthy C, Wormald MR, Casey M, Gabillard-Lefort C, McElvaney NG, Reeves EP.
Int J Mol Sci. 2022 Feb 23;23(5):2441. doi: 10.3390/ijms23052441.
PMID: 35269582 Free PMC article. Review.

Prevalence of Alpha-1 Antitrypsin Deficiency, Self-Reported Behavior Change, and Health Care Engagement Among Direct-to-Consumer Recipients of a Personalized Genetic Risk Report.
Ashenhurst JR, Nhan H, Shelton JF, Wu S, Tung JY, Elson SL, Stoller JK; 23andMe Research Team.
Chest. 2022 Feb;161(2):373-381. doi: 10.1016/j.chest.2021.09.041. Epub 2021 Oct 14.
PMID: 34656525

Site-Specific Conjugation of a Selenopolypeptide to Alpha-1-antitrypsin Enhances Oxidation Resistance and Pharmacological Properties.
Dong C, Wu G, Chen C, Li X, Yuan R, Xu L, Guo H, Zhang J, Lu H, Wang F. Angew Chem Int Ed Engl. 2022 Feb 1;61(6):e202115241. doi: 10.1002/anie.202115241. Epub 2021 Dec 23.
PMID: 34897938

Pregnancy in Alpha 1 Antitrypsin (AAT) Deficiency and the role of intravenous AAT therapy.
Guarnieri G, Achille A, Lococo S, Vianello A.
Pulmonology. 2022 Feb 24:S2531-0437(22)00025-3. doi: 10.1016/j.pulmoe.2022.01.014. Online ahead of print.
PMID: 35221261 No abstract available.

Alpha-1 antitrypsin deficiency-associated panniculitis: A potentially challenging diagnosis but an important one for patients.
Steele L, Mitchell CD, Shipman AR, Shipman KE.
J Am Acad Dermatol. 2022 Feb;86(2):e67-e68. doi: 10.1016/j.jaad.2021.06.897. Epub 2021 Jul 31. PMID: 34343603 No abstract available.

The Relationship between Plasma Alpha-1-Antitrypsin Polymers and Lung or Liver Function in ZZ Alpha-1-Antitrypsin-Deficient Patients.
Sark AD, Fromme M, Olejnicka B, Welte T, Strnad P, Janciauskiene S, Stolk J. Biomolecules. 2022 Feb 28;12(3):380. doi: 10.3390/biom12030380.
PMID: 35327571 Free PMC article.

Term pregnancy of a patient with severe pulmonary emphysema associated with (PI*ZZ) alpha-1 antitrypsin.
Martínez-González C, Adanez Garcia J, Blanco I.
Arch Bronconeumol. 2022 Feb 12:S0300-2896(22)00094-1. doi: 10.1016/j.arbres.2022.02.004. Online ahead of print.
PMID: 35312536 English, Spanish. No abstract available.

January 2022 Publications

Alpha 1 Antitrypsin Deficiency. Meseeha M, Attia M.
2021 Jul 25. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan–.
PMID: 28723059 Free Books & Documents.

The Clinical Course of Severe Alpha-1-Antitrypsin Deficiency in Patients Identified by Screening. Hiller AM, Piitulainen E, Tanash H.
Int J Chron Obstruct Pulmon Dis. 2022 Jan 5;17:43-52. doi: 10.2147/COPD.S340241. eCollection 2022.
PMID: 35023912 Free PMC article.

Alpha 1 Antitrypsin Mutation. Abdulkarim A, Craig TJ.
2021 Jul 18. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan–.
PMID: 29489299 Free Books & Documents.

Leave no one behind: inclusion of alpha-1 antitrypsin deficiency patients in COVID-19 vaccine trials.
Yang C, Zhao H, Tebbutt SJ.
Eur J Hum Genet. 2022 Jan 28:1-3. doi: 10.1038/s41431-022-01047-4. Online ahead of print. PMID: 35087186 Free PMC article.

Production and characterization of mono-PEGylated alpha-1 antitrypsin for augmentation therapy.
Liu X, Vanvarenberg K, Kouassi KGW, Mahri S, Vanbever R.
Int J Pharm. 2022 Jan 25;612:121355. doi: 10.1016/j.ijpharm.2021.121355. Epub 2021 Dec 6.
PMID: 34883205

Designing Clinical Trials in “Regular” COPD Versus Alpha-1 Antitrypsin Deficiency-Associated COPD: “More Alike Than Unalike?”.
Stoller JK.
Chronic Obstr Pulm Dis. 2022 Jan 27;9(1):95-102. doi: 10.15326/jcopdf.2021.0261. PMID: 34735756 Free PMC article.

Alpha-1 antitrypsin deficiency is significantly associated with atopy in asthmatic patients.
Aiello M, Frizzelli A, Pisi R, Fantin A, Ghirardini M, Marchi L, Ferrarotti I, Bertorelli G, Percesepe A, Chetta A.
J Asthma. 2022 Jan;59(1):23-30. doi: 10.1080/02770903.2020.1827421. Epub 2020 Oct 19. PMID: 32962473

A Novel Detection Method to Identify Individuals with Alpha-1 Antitrypsin Deficiency: Linking Prescription of COPD Medications with the Patient-Facing Electronic Medical Record.
Lam SW, Strange C, Brantly ML, Stoller JK.
Chronic Obstr Pulm Dis. 2022 Jan 27;9(1):26-33. doi: 10.15326/jcopdf.2021.0260. PMID: 34784453 Free PMC article.

Alpha-1 antitrypsin deficiency and risk of lung cancer in never-smokers: a multicentre case-control study.
Tubío-Pérez RA, Torres-Durán M, García-Rodríguez ME, Candal-Pedreira C, Rey-Brandariz J, Pérez-Ríos M, Barros-Dios J, Fernández-Villar A, Ruano-Raviña A.
BMC Cancer. 2022 Jan 19;22(1):81. doi: 10.1186/s12885-022-09190-3.
PMID: 35045822 Free PMC article.

α1 -Antitrypsin Z allele and risk of venous thromboembolism in the general population.
Riis J, Nordestgaard BG, Afzal S.
J Thromb Haemost. 2022 Jan;20(1):115-125. doi: 10.1111/jth.15556. Epub 2021 Oct 30. PMID: 34662507

Proteoform Profiles Reveal That Alpha-1-Antitrypsin in Human Serum and Milk Is Derived From a Common Source.
Jager S, Cramer DAT, Hoek M, Mokiem NJ, van Keulen BJ, van Goudoever JB, Dingess KA, Heck
AJR.
Front Mol Biosci. 2022 Feb 22;9:858856. doi: 10.3389/fmolb.2022.858856. eCollection 2022. PMID: 35274008 Free PMC article.

Augmentation Therapy with Alpha-1 Antitrypsin: Present and Future of Production, Formulation, and Delivery.
Bianchera A, Alomari E, Bruno S.
Curr Med Chem. 2022;29(3):385-410. doi: 10.2174/0929867328666210525161942. PMID: 34036902 Review.

Opinions and Attitudes of Pulmonologists About Augmentation Therapy in Patients with Alpha-1 Antitrypsin Deficiency. A Survey of the EARCO Group.
Greulich T, Albert A, Cassel W, Boeselt T, Peychev E, Klemmer A, Ferreira F, Clarenbach C, Torres-Duran ML, Turner AM, Miravitlles M.
Int J Chron Obstruct Pulmon Dis. 2022 Jan 5;17:53-64. doi: 10.2147/COPD.S346051. eCollection 2022.
PMID: 35023913 Free PMC article.

Assessment of Patient Perspectives and Barriers to Self-Infusion of Augmentation Therapy for Alpha-1 Antitrypsin Deficiency During the COVID-19 Pandemic.
Colello J, Ptasinski A, Zhan X, Kaur S, Craig T.
Pulm Ther. 2022 Mar;8(1):95-103. doi: 10.1007/s41030-022-00182-z. Epub 2022 Jan 24.
PMID: 35067906 Free PMC article.

Matrix metalloproteinase-13 is fully activated by neutrophil elastase and inactivates its serpin inhibitor, alpha-1 antitrypsin: Implications for osteoarthritis.
Wilkinson DJ, Falconer AMD, Wright HL, Lin H, Yamamoto K, Cheung K, Charlton SH, Arques MDC, Janciauskiene S, Refaie R, Rankin KS, Young DA, Rowan AD.
FEBS J. 2022 Jan;289(1):121-139. doi: 10.1111/febs.16127. Epub 2021 Jul 30. PMID: 34270864

December 2021 Publications

Alpha-1 antitrypsin deficiency is under-recognized in individuals with cirrhosis undergoing liver transplantation.
Shah RS, Alsuleiman B, Bena J, Stoller JK, Wakim-Fleming J.
Eur J Gastroenterol Hepatol. 2021 Dec 1;33(1S Suppl 1):e233-e238. doi: 10.1097/MEG.0000000000002005.
PMID: 33252420

Maternal alpha-1-antitrypsin as a noval marker for growth restriction in pre-eclampsia.
Nori W, Ali AI.
J Obstet Gynaecol Res. 2021 Dec;47(12):4250-4255. doi: 10.1111/jog.15043. Epub 2021 Sep 27.
PMID: 34571571

Relationship between alpha-1 antitrypsin deficiency and obstructive sleep apnea.
Megenhardt L, Ransdell S, Clark-LoCascio J, Pleasants RA, Brantly M, Strange C.
Sleep Breath. 2021 Dec;25(4):2091-2097. doi: 10.1007/s11325-021-02386-0. Epub 2021 Apr 30.
PMID: 33931809

Lung Function and Health Status in Individuals with Severe Alpha-1-Antitrypsin Deficiency at the Age of 42.
Schramm GR, Mostafavi B, Piitulainen E, Wollmer P, Tanash HA.
Int J Chron Obstruct Pulmon Dis. 2021 Dec 23;16:3477-3485. doi: 10.2147/COPD.S335683. eCollection 2021.
PMID: 34992356 Free PMC article.

The Mechanism of Mitochondrial Injury in Alpha-1 Antitrypsin Deficiency Mediated Liver Disease.
Khodayari N, Wang RL, Oshins R, Lu Y, Millett M, Aranyos AM, Mostofizadeh S, Scindia Y, Flagg TO, Brantly M.
Int J Mol Sci. 2021 Dec 9;22(24):13255. doi: 10.3390/ijms222413255.
PMID: 34948056 Free PMC article.

C3d Elicits Neutrophil Degranulation and Decreases Endothelial Cell Migration, with Implications for Patients with Alpha-1 Antitrypsin Deficiency.
Fee LT, Gogoi D, O’Brien ME, McHugh E, Casey M, Gough C, Murphy M, Hopkins AM, Carroll TP, McElvaney NG, Reeves EP.
Biomedicines. 2021 Dec 16;9(12):1925. doi: 10.3390/biomedicines9121925.
PMID: 34944741 Free PMC article.

SepA Enhances Shigella Invasion of Epithelial Cells by Degrading Alpha-1 Antitrypsin and Producing a Neutrophil Chemoattractant.
Meza-Segura M, Birtley JR, Maldonado-Contreras A, Mueller C, Simin KJ, Stern LJ, McCormick BA.
mBio. 2021 Dec 21;12(6):e0283321. doi: 10.1128/mBio.02833-21. Epub 2021 Nov 2.
PMID: 34724811 Free PMC article.

Proteomic Analysis of Exosomes Secreted from Human Alpha-1 Antitrypsin Overexpressing Mesenchymal Stromal Cells.
Wei H, Green E, Ball L, Fan H, Lee J, Strange C, Wang H.
Biology (Basel). 2021 Dec 21;11(1):9. doi: 10.3390/biology11010009.
PMID: 35053007

Site-Specific Conjugation of a Selenopolypeptide to Alpha-1-antitrypsin Enhances Oxidation Resistance and Pharmacological Properties.
Dong C, Wu G, Chen C, Li X, Yuan R, Xu L, Guo H, Zhang J, Lu H, Wang F.
Angew Chem Int Ed Engl. 2021 Dec 12:e202115241. doi: 10.1002/anie.202115241. Online ahead
of print.
PMID: 34897938

In vitro and in vivo modulation of NADPH oxidase activity and reactive oxygen species production in human neutrophils by α1-antitrypsin.
Hawkins P, McEnery T, Gabillard-Lefort C, Bergin DA, Alfawaz B, Shutchaidat V, Meleady P, Henry M, Coleman O, Murphy M, McElvaney NG, Reeves EP.
ERJ Open Res. 2021 Dec 6;7(4):00234-2021. doi: 10.1183/23120541.00234-2021. eCollection 2021 Oct.
PMID: 34881324 Free PMC article.

P.150: Alpha-1 Antitrypsin Engineered Mesenchymal Stromal Cells Improves Human Islet Survival via Regulation of Macrophage Activation.
Wang H.
Transplantation. 2021 Dec 1;105(12S1):S62. doi: 10.1097/01.tp.0000804664.76961.8d.
PMID: 34908278 No abstract available.

November 2021 Publications

The emerging role of proteases in α1-antitrypsin deficiency and beyond.
Fazleen A, Wilkinson T.
ERJ Open Res. 2021 Nov 22;7(4):00494-2021. doi: 10.1183/23120541.00494-2021. eCollection 2021 Oct.
PMID: 34820446 Free PMC article. Review.

Alpha 1 Antitrypsin Therapy in Patients with Alpha 1 Antitrypsin Deficiency: Perspectives from a Registry Study and Practical Considerations for Self-Administration During the COVID-19 Pandemic.
Herth FJF, Sandhaus RA, Turner AM, Sucena M, Welte T, Greulich T.
Int J Chron Obstruct Pulmon Dis. 2021 Nov 1;16:2983-2996. doi: 10.2147/COPD.S325211. eCollection 2021.
PMID: 34754184 Free PMC article. Review.

The need for continuous quality assessment for providing optimal comprehensive care for patients with alpha-1 antitrypsin deficiency.
Ptasinski A, Colello J, Ptasinski J, Barclay G, Craig T.
Allergy Asthma Proc. 2021 Nov 1;42(6):537-542. doi: 10.2500/aap.2021.42.210067. PMID: 34871162 Free PMC article.

Investigating the Link between Alpha-1 Antitrypsin Deficiency and Abdominal Aortic Aneurysms.
Pini L, Peroni M, Zanotti C, Pini A, Bossoni E, Giordani J, Bargagli E, Perger E, Ferrarotti I, Vizzardi E, Tiberio L, Bonardelli S, Tantucci C.
Ann Vasc Surg. 2021 Nov;77:195-201. doi: 10.1016/j.avsg.2021.05.064. Epub 2021 Aug 26.
PMID: 34455044

Alpha-1 antitrypsin deficiency: a re-surfacing adult liver disorder.
Fromme M, Schneider CV, Trautwein C, Brunetti-Pierri N, Strnad P.
J Hepatol. 2021 Nov 27:S0168-8278(21)02227-3. doi: 10.1016/j.jhep.2021.11.022. Online ahead of print.
PMID: 34848258 Review.

Alpha-1 Antitrypsin MZ Heterozygosity is an Endotype of Chronic Obstructive Pulmonary Disease.
Ghosh AJ, Hobbs BD, Moll M, Saferali A, El-Boueiz A, Yun JH, Sciurba F, Barwick L, Limper AH, Flaherty K, Criner G, Brown KK, Wise R, Martinez FJ, Lomas D, Castaldi PJ, Carey VJ, DeMeo DL, Cho MH, Silverman EK, Hersh CP.
Am J Respir Crit Care Med. 2021 Nov 11. doi: 10.1164/rccm.202106-1404OC. Online ahead of print.
PMID: 34762809

Designing Clinical Trials in “Regular” COPD Versus Alpha-1 Antitrypsin Deficiency-Associated COPD: “More Alike than Unalike?”.
Stoller JK.
Chronic Obstr Pulm Dis. 2021 Nov 3. doi: 10.15326/jcopdf.2021.0261. Online ahead of print.
PMID: 34735756

Adenine base editing reduces misfolded protein accumulation and toxicity in alpha-1 antitrypsin deficient patient iPSC-hepatocytes.
Werder RB, Kaserman JE, Packer MS, Lindstrom-Vautrin J, Villacorta-Martin C, Young LE, Aratyn-Schaus Y, Gregoire F, Wilson AA.
Mol Ther. 2021 Nov 3;29(11):3219-3229. doi: 10.1016/j.ymthe.2021.06.021. Epub 2021 Jul 2.
PMID: 34217893

A Novel Cellular Therapy to Treat Pancreatic Pain in Experimental Chronic Pancreatitis Using Human Alpha-1 Antitrypsin Overexpressing Mesenchymal Stromal Cells.
Chow RP, Nguyen K, Gou W, Green E, Morgan K, Lancaster W, Helke K, Strange C, Wang H. Biomedicines. 2021 Nov 16;9(11):1695. doi: 10.3390/biomedicines9111695.
PMID: 34829924 Free PMC article.

A Novel Detection Method to Identify Individuals with Alpha-1 Antitrypsin Deficiency: Linking Prescription of COPD Medications with the Patient-Facing Electronic Medical Record.
Lam SW, Strange C, Brantly ML, Stoller JK.
Chronic Obstr Pulm Dis. 2021 Nov 16. doi: 10.15326/jcopdf.2021.0260. Online ahead of print.
PMID: 34784453

Is it possible to use the serum levels of alpha 1-antitrypsin as a serum biomarker to distinguish endometriosis and endometriosis-associated epithelial ovarian cancers?
Li YT, Lee WL, Wang PH.
J Chin Med Assoc. 2021 Nov 1;84(11):985-986. doi: 10.1097/JCMA.0000000000000644.
PMID: 34698690 No abstract available.

Correction to: Association between circulating alpha-1 antitrypsin polymers and lung and liver disease.
Núñez A, Belmonte I, Miranda E, Barrecheguren M, Farago G, Loeb E, Pons M, Rodríguez-Frías F, Gabriel-Medina P, Rodríguez E, Genescà J, Miravitlles M, Esquinas C.
Respir Res. 2021 Nov 1;22(1):283. doi: 10.1186/s12931-021-01874-x.
PMID: 34719403 Free PMC article. No abstract available.

October 2021 Publications

Prevalence of Alpha-1 Antitrypsin Deficiency, Self-Reported Behavior Change, and Health Care Engagement Among Direct-to-Consumer Recipients of a Personalized Genetic Risk Report.
Ashenhurst JR, Nhan H, Shelton JF, Wu S, Tung JY, Elson SL, Stoller JK; 23andMe Research Team. Chest. 2021 Oct 14:S0012-3692(21)04099-X. doi: 10.1016/j.chest.2021.09.041. Online ahead of print.
PMID: 34656525

Relationship between Depression and Anxiety, Health Status and Lung Function in Patients with Alpha-1 Antitrypsin Deficiency.
Mobeen F, Edgar RG, Pye A, Stockley RA, Turner AM.
COPD. 2021 Oct 22:1-9. doi: 10.1080/15412555.2021.1991904. Online ahead of print.
PMID: 34676796

IntraIndividual Variability in Serum Alpha-1 Antitrypsin Levels.
Haillot A, Pelland AA, Bossé Y, Carroll TP, Maltais F, Dandurand RJ.
Chronic Obstr Pulm Dis. 2021 Oct 28;8(4):464-473. doi: 10.15326/jcopdf.2021.0228. PMID: 34407569
Free PMC article.

Association of the methionine sulfoxide reductase A rs10903323 gene polymorphism with functional activity and oxidative modification of alpha-1-antitrypsin in COPD patients.
Milovanovic V, Topic A, Milinkovic N, Lazic Z, Ivosevic A, Radojkovic D, Rankov AD. Pulmonology. 2021 Oct 18:S2531-0437(21)00193-8. doi: 10.1016/j.pulmoe.2021.09.003. Online ahead of print.
PMID: 34674978

September 2021 Publications

Association between circulating alpha-1 antitrypsin polymers and lung and liver disease.
Núñez A, Belmonte I, Miranda E, Barrecheguren M, Farago G, Loeb E, Pons M, Rodríguez-Frías F, Gabriel-Medina P, Rodríguez E, Genescà J, Miravitlles M, Esquinas C.
Respir Res. 2021 Sep 15;22(1):244. doi: 10.1186/s12931-021-01842-5.
PMID: 34526035 Free PMC article.

Alpha-1 Antitrypsin and Hepatocellular Carcinoma in Liver Cirrhosis: SERPINA1 MZ or MS Genotype Carriage Decreases the Risk.
Rabekova Z, Frankova S, Jirsa M, Neroldova M, Lunova M, Fabian O, Kveton M, Varys D, Chmelova K, Adamkova V, Hubacek JA, Spicak J, Merta D, Sperl J.
Int J Mol Sci. 2021 Sep 29;22(19):10560. doi: 10.3390/ijms221910560.
PMID: 34638908 Free PMC article.

Maternal alpha-1-antitrypsin as a noval marker for growth restriction in pre-eclampsia.
Nori W, Ali AI.
J Obstet Gynaecol Res. 2021 Sep 27. doi: 10.1111/jog.15043. Online ahead of print. PMID: 34571571

Estimated Prevalence and Number of PiMZ Genotypes of Alpha-1 Antitrypsin in Seventy-Four Countries Worldwide.
Martinez-González C, Blanco I, Diego I, Bueno P, Miravitlles M.
Int J Chron Obstruct Pulmon Dis. 2021 Sep 17;16:2617-2630. doi: 10.2147/COPD.S327803. eCollection 2021.
PMID: 34556982 Free PMC article.

Transgenic Mice Overexpressing Human Alpha-1 Antitrypsin Exhibit Low Blood Pressure and Altered Epithelial Transport Mechanisms in the Inactive and Active Cycles.
Liu LP, Gholam MF, Elshikha AS, Kawakibi T, Elmoujahid N, Moussa HH, Song S, Alli AA. Front Physiol. 2021 Sep 22;12:710313. doi: 10.3389/fphys.2021.710313. eCollection 2021.
PMID: 34630137 Free PMC article.

Is the diagnostic rate for the common subtypes of A1AT deficiency consistent across two Canadian Provinces?
Estey MP, Tahooni T, Nelson TN, Parker ML, Agbor TA, Yang HM, Jen R, Barakauskas VE, Lam GY, Matthews A, Mattman A.
Clin Biochem. 2021 Sep;95:84-88. doi: 10.1016/j.clinbiochem.2021.05.002. Epub 2021 May 6. PMID: 33964271

Pulmonary transplantation of alpha-1 antitrypsin (AAT)-transgenic macrophages provides a source of functional human AAT in vivo.
Janosz E, Hetzel M, Spielmann H, Tumpara S, Rossdam C, Schwabbauer M, Kloos D, von Kaisenberg C, Schambach A, Buettner FFR, Janciauskiene S, Lachmann N, Moritz T. Gene Ther. 2021 Sep;28(9):477-493. doi: 10.1038/s41434-021-00269-3. Epub 2021 Jul 19.
PMID: 34276045 Free PMC article. Review.

Alpha-1 Antitrypsin Z Variant (AAT PI*Z) as a Risk Factor for Intrahepatic Cholestasis of Pregnancy.
Kosinski P, Kedzia M, Mostowska A, Gutaj P, Lipa M, Wender-Ozegowska E, Rozy A, Chorostowska-Wynimko J, Wielgos M, Jezela-Stanek A.
Front Genet. 2021 Sep 7;12:720465. doi: 10.3389/fgene.2021.720465. eCollection 2021. PMID: 34557220 Free PMC article.

A Particular SORL1 Micro-haplotype May Prevent Severe Liver Disease in a French Cohort of Alpha 1-Antitrypsin-deficient Children.
Joly P, Ruiz M, Garin R, Karatas E, Lachaux A, Restier L, Belmalih A, Renoux C, Lombard C, Dechomet M, Bouchecareilh M.
J Pediatr Gastroenterol Nutr. 2021 Sep 1;73(3):e68-e72. doi: 10.1097/MPG.0000000000003125. PMID: 33720088

August 2021 Publications

Heterozygosity of the Alpha 1-Antitrypsin Pi*Z Allele and Risk of Liver Disease.
Hakim A, Moll M, Qiao D, Liu J, Lasky-Su JA, Silverman EK, Vilarinho S, Jiang ZG, Hobbs BD, Cho MH.
Hepatol Commun. 2021 Apr 3;5(8):1348-1361. doi: 10.1002/hep4.1718. eCollection 2021 Aug. PMID: 34430780 Free PMC article.

Hepatocyte proteomes reveal the role of protein disulfide isomerase 4 in alpha 1-antitrypsin deficiency.
Karatas E, Raymond AA, Leon C, Dupuy JW, Di-Tommaso S, Senant N, Collardeau-Frachon S, Ruiz M, Lachaux A, Saltel F, Bouchecareilh M.
JHEP Rep. 2021 Apr 24;3(4):100297. doi: 10.1016/j.jhepr.2021.100297. eCollection 2021 Aug. PMID: 34151245 Free PMC article.

Investigating the Link between Alpha-1 Antitrypsin Deficiency and Abdominal Aortic Aneurysms.
Pini L, Peroni M, Zanotti C, Pini A, Bossoni E, Giordani J, Bargagli E, Perger E, Ferrarotti I, Vizzardi E, Tiberio L, Bonardelli S, Tantucci C.
Ann Vasc Surg. 2021 Aug 26:S0890-5096(21)00548-3. doi: 10.1016/j.avsg.2021.05.064. Online
ahead of print. PMID: 34455044

Protective role of the alpha-1-antitrypsin in intervertebral disc degeneration.
Liu W, Wang Y.
J Orthop Surg Res. 2021 Aug 20;16(1):516. doi: 10.1186/s13018-021-02668-z.
PMID: 34416893 Free PMC article.

Alpha-1 Antitrypsin Augmentation Inhibits Proteolysis of Neutrophil Membrane Voltage-Gated Proton Channel-1 in Alpha-1 Deficient Individuals.
Hawkins P, Sya J, Hup NK, Murphy MP, McElvaney NG, Reeves EP. Medicina (Kaunas). 2021 Aug 10;57(8):814. doi: 10.3390/medicina57080814.
PMID: 34441020 Free PMC article.

Intra-Individual Variability in Serum Alpha-1 Antitrypsin Levels.
Haillot A, Pelland AA, Bossé Y, Carroll TP, Maltais F, Dandurand RJ.
Chronic Obstr Pulm Dis. 2021 Aug 17. doi: 10.15326/jcopdf.2021.0228. Online ahead of print. PMID: 34407569

Long-term effect of α1-antitrypsin augmentation therapy on the decline of FEV1 in deficient patients: an analysis of the AIR database.
Schouten IGM, Kasteleyn MJ, Tsonaka R, Bals R, Turner AC, Ferrarotti I, Corsico AG, Lara B, Miravitlles M, Stockley RA, Stolk J.
ERJ Open Res. 2021 Aug 23;7(3):00194-2021. doi: 10.1183/23120541.00194-2021. eCollection 2021 Jul.
PMID: 34435030 Free PMC article.

Quantitative measurement of the histological features of alpha-1 antitrypsin deficiency-associated liver disease in biopsy specimens.
Marek G, Collinsworth A, Liu C, Brantly M, Clark V.
PLoS One. 2021 Aug 16;16(8):e0256117. doi: 10.1371/journal.pone.0256117. eCollection 2021. PMID: 34398915 Free PMC article.

The Induction of Alpha-1 Antitrypsin by Vitamin D in Human T Cells Is TGF-β Dependent: A Proposed Anti-inflammatory Role in Airway Disease.
Chen YH, Cheadle CE, Rice LV, Pfeffer PE, Dimeloe S, Gupta A, Bush A, Gooptu B, Hawrylowicz CM.
Front Nutr. 2021 Aug 12;8:667203. doi: 10.3389/fnut.2021.667203. eCollection 2021. PMID: 34458299 Free PMC article.

In Vitro Investigations on Optimizing and Nebulization of IVT-mRNA Formulations for Potential Pulmonary-Based Alpha-1-Antitrypsin Deficiency Treatment.
Guan S, Darmstädter M, Xu C, Rosenecker J.
Pharmaceutics. 2021 Aug 17;13(8):1281. doi: 10.3390/pharmaceutics13081281. PMID: 34452241 Free PMC article.

July 2021 Publications

Alpha 1 Antitrypsin Deficiency. Meseeha M, Attia M.
2021 Jul 25. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan–.
PMID: 28723059 Free Books & Documents. Review.

[Alpha-1-antitrypsin deficiency].
de Vos JD, Hillberg O, Perch M, Jensen JU, Wilcke JT, Løkke A. Ugeskr Laeger. 2021 Jul 26;183(30):V02210150.
PMID: 34356027 Review. Danish.

Alpha 1 Antitrypsin Mutation.
Abdulkarim A, Craig TJ. 2021 Jul 18. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan–.
PMID: 29489299 Free Books & Documents. Review.

COVID-19 infection in severe Alpha 1-antitrypsin deficiency: Looking for a rationale.
Ferrarotti I, Ottaviani S, Balderacchi AM, Barzon V, De Silvestri A, Piloni D, Mariani F, Corsico AG.
Respir Med. 2021 Jul;183:106440. doi: 10.1016/j.rmed.2021.106440. Epub 2021 Apr 30. PMID: 33964815 Free PMC article. Review.

Imaging in alpha-1 antitrypsin deficiency: a window into the disease. Huang YT, Wencker M, Driehuys B.
Ther Adv Chronic Dis. 2021 Jul 29;12_suppl:20406223211024523. doi: 10.1177/20406223211024523. eCollection 2021.
PMID: 34408834 Free PMC article. Review.

Alpha-1 antitrypsin deficiency research and emerging treatment strategies: what’s down the road? Rahaghi FF.
Ther Adv Chronic Dis. 2021 Jul 29;12_suppl:20406223211014025. doi: 10.1177/20406223211014025. eCollection 2021.
PMID: 34408832 Free PMC article. Review.

Alpha-1 antitrypsin deficiency hidden in allegedly normal variants.
Suárez-Lorenzo I, Hernández-Brito E, Almeida-Quintana L, Llanos CG, González-Quevedo N, Carrillo-Díaz T, Rodríguez-Gallego C.
J Asthma. 2021 Jul 13:1-4. doi: 10.1080/02770903.2021.1944186. Online ahead of print. PMID: 34152895

The spectrum of clinical sequelae associated with alpha-1 antitrypsin deficiency. Tejwani V, Stoller JK.
Ther Adv Chronic Dis. 2021 Jul 29;12_suppl:2040622321995691. doi: 10.1177/2040622321995691. eCollection 2021.
PMID: 34408829 Free PMC article. Review.

Association of Alpha 1-antitrypsin Deficiency and Genetic Predisposition in Primary Spontaneous Pneumothorax.
Sahin T, Ozsoy IE, Tezcan MA, Kocer D, Erdogan M.
J Coll Physicians Surg Pak. 2021 Jul;30(7):775-779. doi: 10.29271/jcpsp.2021.07.775. PMID: 34271775

Lung and liver transplantation in patients with alpha-1 antitrypsin deficiency. Zamora MR, Ataya A.
Ther Adv Chronic Dis. 2021 Jul 29;12_suppl:20406223211002988. doi: 10.1177/20406223211002988. eCollection 2021.
PMID: 34408830 Free PMC article. Review.

Variants of SERPINA1 and the increasing complexity of testing for alpha-1 antitrypsin deficiency. Foil KE.
Ther Adv Chronic Dis. 2021 Jul 29;12_suppl:20406223211015954. doi: 10.1177/20406223211015954. eCollection 2021.
PMID: 34408833 Free PMC article. Review.

Association of α 1 Antitrypsin Phenotype and Development of Advanced Liver Disease and Pulmonary Complications Before and After Liver Transplantation.
Abu Rmilah A, Fencl R, Watt K, Krowka M, Wiesner R, Murray D, Nyberg S, Leise M. Transplantation. 2021 Jul 1;105(7):1576-1584. doi: 10.1097/TP.0000000000003390.
PMID: 32732616

Management of lung disease in alpha-1 antitrypsin deficiency: what we do and what we do not know.
Barjaktarevic I, Campos M. Ther Adv Chronic Dis. 2021 Jul 29;12_suppl:20406223211010172. doi: 10.1177/20406223211010172. eCollection 2021.
PMID: 34408831 Free PMC article. Review.

Prevalence and clinical characteristics of alpha-1 antitrypsin deficiency in liver explants in a Mexican cohort.
Campos-Murguía A, Valdéz-Hernández P, Cordova-Gallardo J, Arteaga-Vázquez J, Contreras AG, Vilatobá M, Cruz-Martínez R, Martínez-Benítez B, Gamboa-Domínguez A, Marfil-Garza BA, Flores-García NC, Márquez-Guillén E, García-Juárez I. Clin Res Hepatol Gastroenterol. 2021 Jul;45(4):101519. doi: 10.1016/j.clinre.2020.07.024. Epub 2021 Feb 23.
PMID: 33636655

Liver disease with unknown etiology – have you ruled out alpha-1 antitrypsin deficiency? Patel D, Teckman J.
Ther Adv Chronic Dis. 2021 Jul 29;12_suppl:2040622321995684. doi: 10.1177/2040622321995684. eCollection 2021.
PMID: 34408828 Free PMC article. Review.

The Course of AαVal541 as a Proteinase 3 Specific Neo-Epitope after Alpha-1-Antitrypsin Augmentation in Severe Deficient Patients.
Schouten IGM, Mumford RA, Moes DJAR, Hiemstra PS, Stolk J. Int J Mol Sci. 2021 Jul 27;22(15):8031. doi: 10.3390/ijms22158031.
PMID: 34360796 Free PMC article. Clinical Trial.

Therapeutic Application of Alpha-1 Antitrypsin in COVID-19.
Ritzmann F, Chitirala P, Krüger N, Hoffmann M, Zuo W, Lammert F, Smola S, Tov N, Alagem N, Lepper PM, Pöhlmann S, Beisswenger C, Herr C, Bals R; AAT-in-COVID-19 Study Group.
Am J Respir Crit Care Med. 2021 Jul 15;204(2):224-227. doi: 10.1164/rccm.202104-0833LE. PMID: 33961754 No abstract available.

Adenine base editing reduces misfolded protein accumulation and toxicity in alpha-1 antitrypsin deficient patient iPSC-hepatocytes.
Werder RB, Kaserman JE, Packer MS, Lindstrom-Vautrin J, Villacorta-Martin C, Young LE, Aratyn-Schaus Y, Gregoire F, Wilson AA.
Mol Ther. 2021 Jul 2:S1525-0016(21)00351-8. doi: 10.1016/j.ymthe.2021.06.021. Online ahead of print.
PMID: 34217893

<<Alpha-1 antitrypsin deficiency-associated panniculitis>> A potentially challenging diagnosis but an important one for patients.
Steele L, Mitchell CD, Shipman AR, Shipman KE.
J Am Acad Dermatol. 2021 Jul 31:S0190-9622(21)02196-4. doi: 10.1016/j.jaad.2021.06.897.
Online ahead of print.
PMID: 34343603 No abstract available

Matrix metalloproteinase-13 is fully activated by neutrophil elastase and inactivates its serpin inhibitor, alpha-1 antitrypsin: Implications for osteoarthritis.
Wilkinson DJ, Falconer AMD, Wright HL, Lin H, Yamamoto K, Cheung K, Charlton SH, Arques MDC, Janciauskiene S, Refaie R, Rankin KS, Young DA, Rowan AD.
FEBS J. 2021 Jul 16. doi: 10.1111/febs.16127. Online ahead of print. PMID: 34270864

June 2021 Publications

Salivary alpha-1-antitrypsin and macrophage migration inhibitory factor may be potential prognostic biomarkers for oncologic treatment-induced severe oral mucositis.
Palmier NR, Leme AFP, De Rossi T, Telles GP, Morais-Faria K, Kowalski LP, Marta GN, Brandão TB, Arany PR, Migliorati CA, Santos-Silva AR, Prado-Ribeiro AC.
Support Care Cancer. 2021 Jun;29(6):2939-2946. doi: 10.1007/s00520-020-05805-2. Epub 2020 Oct 2.
PMID: 33009579

Severe alpha-1-antitrypsin deficiency increases the risk of venous thromboembolism.
Basil N, Ekström M, Piitulainen E, Lindberg A, Rönmark E, Jehpsson L, Tanash H. J Thromb Haemost.
2021 Jun;19(6):1519-1525. doi: 10.1111/jth.15302. Epub 2021 Apr 9. PMID: 33763945

Alpha-1 antitrypsin deficiency: clarifying the role of the putative protective threshold.
Franciosi AN, Fraughen D, Carroll TP, McElvaney NG. Eur Respir J.
2021 Jun 25:2101410. doi: 10.1183/13993003.01410-2021. Online ahead of print.
PMID: 34172471

[Alpha-1 antitrypsin deficiency: cause and cofactor for liver disease].
Burbaum B, Fromme M, Strnad P.
Dtsch Med Wochenschr. 2021 Jun;146(11):714-718. doi: 10.1055/a-1277-9066. Epub 2021 Jun 1.
PMID: 34062584 German.

Relationship Between α1-Antitrypsin Deficiency and Ascending Aortic Distention.
Dako F, Zhao H, Mulvenna A, Gupta YS, Simpson S, Kueppers F. Mayo Clin Proc Innov Qual Outcomes. 2021 Apr 30;5(3):590-595. doi: 10.1016/j.mayocpiqo.2021.03.004. eCollection 2021 Jun. PMID: 34195551 Free PMC article.

Alpha-1 antitrypsin deficiency hidden in allegedly normal variants.
Suárez-Lorenzo I, Hernández-Brito E, Almeida-Quintana L, García-de Llanos C, González- Quevedo N, Carrillo-Díaz T, Rodríguez-Gallego C. J Asthma. 2021 Jun 21:1-5. doi: 10.1080/02770903.2021.1944186. Online ahead of print. PMID: 34152895

Placental HTRA1 cleaves alpha-1-antitrypsin to generate a NET-Inhibitory Peptide. Campbell RA, Campbell HD, Bircher JS, de Araujo CV, Denorme F, Crandell JL, Rustad JL, Monts J, Cody MJ, Kosaka Y, Yost CC. Blood. 2021 Jun 30:blood.2020009021. doi: 10.1182/blood.2020009021. Online ahead of print. PMID: 34192300

The development of highly potent and selective small molecule correctors of Z α1-antitrypsin misfolding.
Liddle J, Pearce AC, Arico-Muendel C, Belyanskaya S, Brewster A, Brown M, Chung CW, Denis A, Dodic N, Dossang A, Eddershaw P, Klimaszewska D, Haq I, Holmes DS, Jagger A, Jakhria T, Jigorel E, Lind K, Messer J, Neu M, Olszewski A, Ronzoni R, Rowedder J, Rüdiger M, Skinner S, Smith KJ, Trottet L, Uings I, Zhu Z, Irving JA, Lomas DA. Bioorg Med Chem Lett. 2021 Jun 1;41:127973. doi: 10.1016/j.bmcl.2021.127973. Epub 2021 Mar 19.
PMID: 33753261

A 28-day clinical trial of aerosolized hyaluronan in alpha-1 antiprotease deficiency COPD using desmosine as a surrogate marker for drug efficacy.
Cantor JO, Ma S, Liu X, Campos MA, Strange C, Stocks JM, Devine MS, El Bayadi SG, Lipchik RJ, Sandhaus RA, Turino GM.
Respir Med. 2021 Jun;182:106402. doi: 10.1016/j.rmed.2021.106402. Epub 2021 Apr 14. PMID: 33906126

Alphataxin, an Orally Available Small Molecule, Decreases LDL Levels in Mice as a Surrogate for the LDL-Lowering Activity of Alpha-1 Antitrypsin in Humans.
Bristow CL, Winston R. Front Pharmacol. 2021 Jun 9;12:695971. doi: 10.3389/fphar.2021.695971. eCollection 2021. PMID: 34177602 Free PMC article.

Serendipitous detection of α1-antitrypsin deficiency: a single institution’s experience over a 32 month period.
Cronin T, Rasheed E, Naughton A, McElvaney NG, Carroll TP, Crowley VEF, Conlon N. Clin Chem Lab Med. 2021 Jan 26;59(7):e293-e295. doi: 10.1515/cclm-2020-1750. Print 2021 Jun 25. PMID: 33544483 No abstract available.

May 2021 Publications

Long term results of liver transplantation for alpha-1 antitrypsin deficiency.
Guillaud O, Jacquemin E, Couchonnal E, Vanlemmens C, Francoz C, Chouik Y, Conti F, Duvoux C, Hilleret MN, Kamar N, Houssel-Debry P, Neau-Cransac M, Pageaux GP, Gonzales E, Ackermann O, Gugenheim J, Lachaux A, Ruiz M, Radenne S, Debray D, Lacaille F, McLin V, Duclos-Vallée JC, Samuel D, Coilly A, Dumortier J. Dig Liver Dis.
2021 May;53(5):606-611. doi: 10.1016/j.dld.2020.10.016. Epub 2020 Nov 1. PMID: 33139195

Alpha-1 antitrypsin suppresses macrophage activation and promotes islet graft survival after intrahepatic islet transplantation.
Gou W, Wang J, Song L, Kim DS, Cui W, Strange C, Wang H. Am J Transplant.
2021 May;21(5):1713-1724. doi: 10.1111/ajt.16342. Epub 2020 Nov 16. PMID: 33047509 Free PMC article.

Augmentation therapy with alpha 1-antitrypsin: present and future of production, formulation, and delivery.
Bianchera A, Alomari E, Bruno S. Curr Med Chem.
2021 May 25. doi: 10.2174/0929867328666210525161942. Online ahead of print. PMID: 34036902

Alpha1-antitrypsin Disease, Treatment and Role for Lung Volume Reduction Surgery. Foray N, Stone T, White P. Thorac Surg Clin.
2021 May;31(2):139-160. doi: 10.1016/j.thorsurg.2021.02.002. PMID: 33926668 Review.

A PI*MS is not always a PI*MS. An example of when genotyping for alpha-1 antitrypsin deficiency is necessary.
Martín T, Miravitlles M, Furtado ST. Pulmonology.
2021 May-Jun;27(3):272-273. doi: 10.1016/j.pulmoe.2020.06.013. Epub 2020 Jul 25. PMID: 32723617 No abstract available.

Alpha 1-Antitrypsin deficiency in liver explants in a Mexican cohort: A unique cohort to assess the role of heterozygous genotypes in liver disease.
Karatas E, Bouchecareilh M. Clin Res Hepatol Gastroenterol. 2021 May;45(3):101538. doi: 10.1016/j.clinre.2020.09.003. Epub 2020 Oct 14.
PMID: 33069636 No abstract available.

Alpha-1-antitrypsin reduces inflammation and exerts chondroprotection in arthritis.
Kaneva MK, Muley MM, Krustev E, Reid AR, Souza PR, Dell’Accio F, McDougall JJ, Perretti M. FASEB J. 2021 May;35(5):e21472. doi: 10.1096/fj.202001801R.
PMID: 33788977

Alpha-1 Antitrypsin Deficiency-Associated Clinical Manifestations and Healthcare Resource Use in the United States.
Herrera EM, Joseph C, Brouwer ES, Gandhi V, Czorniak M. COPD.
2021 May 26:1-10. doi: 10.1080/15412555.2021.1917532. Online ahead of print. PMID: 34036848

Update on and future perspectives for the diagnosis of alpha-1 antitrypsin deficiency in Brazil. Jardim JR, Casas-Maldonado F, Fernandes FLA, Castellano MVCO, Torres-Durán M, Miravitlles M. J Bras Pneumol. 2021 May 31;47(3):e20200380. doi: 10.36416/1806-3756/e20200380. eCollection 2021.
PMID: 34076174 English, Portuguese.

The effects of non-surgical periodontal therapy on neutrophil elastase and elastase alpha-1 proteinase inhibitor levels in GCF in periodontitis patients with or without acute coronary syndrome.
Kayar NA, Üstün K, Gözlü M, Haliloğlu S, Alptekin NÖ. Clin Oral Investig. 2021 May;25(5):3329-3338. doi: 10.1007/s00784-021-03838-3. Epub 2021 Mar 9.
PMID: 33687554

Alpha-1 antitrypsin deficiency in the elderly: a case report.
Annunziata A, Lanza M, Coppola A, Fiorentino G. J Med Case Rep. 2021 May 10;15(1):231. doi: 10.1186/s13256-021-02847-w.
PMID: 33966640 Free PMC article.

Polymerization of misfolded Z alpha-1 antitrypsin protein lowers CX3CR1 expression in human PBMCs.
Tumpara S, Ballmaier M, Wrenger S, König M, Lehmann M, Lichtinghagen R, Martinez-Delgado B, Korenbaum E, DeLuca D, Jedicke N, Welte T, Fromme M, Strnad P, Stolk J, Janciauskiene S. Elife. 2021 May 18;10:e64881. doi: 10.7554/eLife.64881.
PMID: 34002692 Free PMC article.

Is the diagnostic rate for the common subtypes of A1AT deficiency consistent across two Canadian Provinces?
Estey MP, Tahooni T, Nelson TN, Parker ML, Agbor TA, Yang HM, Jen R, Barakauskas VE, Lam GY, Matthews A, Mattman A. Clin Biochem. 2021 May 6:S0009-9120(21)00143-0. doi: 10.1016/j.clinbiochem.2021.05.002. Online ahead of print.
PMID: 33964271

The Importance of N186 in the Alpha-1-Antitrypsin Shutter Region Is Revealed by the Novel Bologna Deficiency Variant.
Ronzoni R, Ferrarotti I, D’Acunto E, Balderacchi AM, Ottaviani S, Lomas DA, Irving JA, Miranda E, Fra A. Int J Mol Sci. 2021 May 26;22(11):5668. doi: 10.3390/ijms22115668.
PMID: 34073489 Free PMC article.

Combined pulmonary fibrosis and emphysema in alpha-1-antitrypsin deficiency.
Le Beller A, Nasser M, Traclet J, Blanchet-Legens AS, Kannengiesser C, Mornex JF, Cottin V. Respir Med Res.
2021 May;79:100819. doi: 10.1016/j.resmer.2021.100819. Epub 2021 Mar 26.
PMID: 33862498 No abstract available.

Alpha-1 antitrypsin deficiency severity and the risk of COVID-19: A Portuguese cohort.
Faria N, Inês Costa M, Gomes J, Sucena M. Respir Med. 2021 May;181:106387. doi: 10.1016/j.rmed.2021.106387. Epub 2021 Apr 2. PMID: 33848921
Free PMC article. No abstract available.

Therapeutic Application of alpha-1-antitrypsin in COVID-19.
Ritzmann F, Chitirala P, Krüger N, Hoffmann M, Zuo W, Lammert F, Smola S, Tov N, Alagem N, Lepper PM, Pöhlmann S, Beisswenger C, Herr C, Bals R; AAT-in-COVID-19 study group. Am J Respir Crit Care Med. 2021 May 7. doi: 10.1164/rccm.202104-0833LE. Online ahead of print.
PMID: 33961754 No abstract available.

April 2021 Publications

The molecular species responsible for α1 -antitrypsin deficiency are suppressed by a small molecule chaperone.
Ronzoni R, Heyer-Chauhan N, Fra A, Pearce AC, Rüdiger M, Miranda E, Irving JA, Lomas DA. FEBS J. 2021 Apr;288(7):2222-2237. doi: 10.1111/febs.15597. Epub 2020 Nov 11.PMID: 33058391

Identification of Variants in Alpha-1-Antitrypsin by High Resolution Melting. Bidla G, Rosenblatt DS, Gilfix BM.
J Appl Lab Med. 2021 Apr 29;6(3):715-728. doi: 10.1093/jalm/jfaa191.
PMID: 33331634

Severe alpha-1-antitrypsin deficiency increases the risk of venous thromboembolism. Basil N, Ekström M, Piitulainen E, Lindberg A, Rönmark E, Jehpsson L, Tanash H.
J Thromb Haemost. 2021 Jun;19(6):1519-1525. doi: 10.1111/jth.15302. Epub 2021 Apr 9.
PMID: 33763945

The Pi∗MZ Allele in Alpha-1 Antitrypsin Increases Liver-Related Outcomes in a Population-Based Study.
Luukkonen PK, Salomaa V, Åberg F. Gastroenterology. 2021 Apr;160(5):1874-1875. doi: 10.1053/j.gastro.2020.10.061. Epub 2020 Dec 29.
PMID: 33385428 No abstract available.

Alpha-1 antitrypsin deficiency liver disease. Patel D, McAllister SL, Teckman JH. Transl Gastroenterol Hepatol. 2021 Apr 5;6:23. doi: 10.21037/tgh.2020.02.23. eCollection 2021.
PMID: 33824927 Free PMC article. Review.

Improving Screening for Alpha-1 Antitrypsin Deficiency with Direct Testing in the Pulmonary Function Testing Laboratory.
Gurevich S, Daya A, Da Silva C, Girard C, Rahaghi F.
Chronic Obstr Pulm Dis. 2021 Apr 27;8(2):190-197. doi: 10.15326/jcopdf.2020.0179.
PMID: 33290644 Free PMC article.

Relationship between alpha-1 antitrypsin deficiency and obstructive sleep apnea. Megenhardt L, Ransdell S, Clark-LoCascio J, Pleasants RA, Brantly M, Strange C. Sleep Breath. 2021 Apr 30. doi: 10.1007/s11325-021-02386-0. Online ahead of print.
PMID: 33931809

Relationship Between α1-Antitrypsin Deficiency and Ascending Aortic Distention. Dako F, Zhao H, Mulvenna A, Gupta YS, Simpson S, Kueppers F.
Mayo Clin Proc Innov Qual Outcomes. 2021 Apr 30;5(3):590-595. doi: 10.1016/j.mayocpiqo.2021.03.004. eCollection 2021 Jun.
PMID: 34195551 Free PMC article.

Association of alpha-1-antitrypsin deficiency with vitamin D status: who is most at risk of getting severe COVID-19?
Shimi G, Zand H. Inflamm Res. 2021 Apr;70(4):375-377. doi: 10.1007/s00011-021-01456-z. Epub 2021 Mar 19.
PMID: 33740066 Free PMC article.

Alpha-1 Antitrypsin-Induced Endoplasmic Reticulum Stress Promotes Invasion by Extravillous Trophoblasts.
Yoshida K, Kusama K, Fukushima Y, Ohmaru-Nakanishi T, Kato K, Tamura K. Int J Mol Sci. 2021 Apr 1;22(7):3683. doi: 10.3390/ijms22073683.
PMID: 33916165 Free PMC article.

A randomised, double-blind, placebo-controlled, pilot trial of intravenous plasma purified alpha-1 antitrypsin for SARS-CoV-2-induced Acute Respiratory Distress Syndrome: a structured summary of a study protocol for a randomised, controlled trial.
McEvoy NL, Clarke JL, Mc Elvaney OJ, Mc Elvaney OF, Boland F, Hyland D, Geoghegan P, Donnelly K, Friel O, Cullen A, Collins AM, Fraughen D, Martin-Loeches I, Hennessy M, Laffey JG, Mc Elvaney NG, Curley GF.
Trials. 2021 Apr 19;22(1):288. doi: 10.1186/s13063-021-05254-0.
PMID: 33874981 Free PMC article.

Therapeutic Potential of Alpha-1 Antitrypsin in Type 1 and Type 2 Diabetes Mellitus.
Park SS, Rodriguez Ortega R, Agudelo CW, Perez Perez J, Perez Gandara B, Garcia-Arcos I, McCarthy C, Geraghty P. Medicina (Kaunas). 2021 Apr 20;57(4):397. doi: 10.3390/medicina57040397.
PMID: 33923873 Free PMC article. Review.

Effect of Alpha-1 Antitrypsin on CFTR Levels in Primary Human Airway Epithelial Cells Grown at the Air-Liquid-Interface.
Stanke F, Janciauskiene S, Tamm S, Wrenger S, Raddatz EL, Jonigk D, Braubach P. Molecules. 2021 Apr 30;26(9):2639. doi: 10.3390/molecules26092639.
PMID: 33946490 Free PMC article.

Alpha-1 Antitrypsin Deficiency: Home Therapy.
Annunziata A, Lanza M, Coppola A, Andreozzi P, Spinelli S, Fiorentino G.
Front Pharmacol. 2021 Apr 15;12:575402. doi: 10.3389/fphar.2021.575402. eCollection 2021.
PMID: 33935692 Free PMC article.

Treatment of α-1 antitrypsin deficiency using hepatic-specified cells derived from human-induced pluripotent stem cells.
Chen Y, Li R, Zhang L, Gan L, Ding J. Am J Transl Res. 2021 Apr 15;13(4):2710-2716. eCollection 2021.
PMID: 34017432 Free PMC article.

Alpha-1 Antitrypsin Screening in a Selected Cohort of Patients Affected by Chronic Pulmonary Diseases in Naples, Italy.
Annunziata A, Ferrarotti I, Coppola A, Lanza M, Imitazione P, Spinelli S, Micco PD, Fiorentino G. J Clin Med. 2021 Apr 7;10(8):1546. doi: 10.3390/jcm10081546.
PMID: 33916947 Free PMC article.

Correlation of Low Levels of α-1 Antitrypsin and Elevation of Neutrophil to Lymphocyte Ratio with Higher Mortality in Severe COVID-19 Patients.
Shimi G, Sohrab G, Pourvali K, Ghorbani A, Balam FH, Rostami K, Zand H. Mediators Inflamm. 2021 Apr 28;2021:5555619. doi: 10.1155/2021/5555619. eCollection 2021. PMID: 34007243 Free PMC article.

Alpha 1 Antitrypsin is an Inhibitor of the SARS-CoV-2-Priming Protease TMPRSS2.
Azouz NP, Klingler AM, Callahan V, Akhrymuk IV, Elez K, Raich L, Henry BM, Benoit JL, Benoit SW, Noé F, Kehn-Hall K, Rothenberg ME.
Pathog Immun. 2021 Apr 26;6(1):55-74. doi: 10.20411/pai.v6i1.408. eCollection 2021.
PMID: 33969249 Free PMC article.

Heterozygous Alpha-1 Antitrypsin Deficiency Causing Pulmonary Emboli and Pulmonary Bullae.
Tannous T, Rosso C, Keating M.Cureus. 2021 Apr 29;13(4):e14759. doi: 10.7759/cureus.14759.
PMID: 34084683 Free PMC article.

Epidemiological correlation between COVID-19 epidemic and prevalence of α-1 antitrypsin deficiency in the world.
Yoshikura H. Glob Health Med. 2021 Apr 30;3(2):73-81. doi: 10.35772/ghm.2020.01068.
PMID: 33937569 Free PMC article.

Alpha-1 antitrypsin deficiency severity and the risk of COVID-19: A Portuguese cohort. Faria N, Inês Costa M, Gomes J, Sucena M.
Respir Med. 2021 May;181:106387. doi: 10.1016/j.rmed.2021.106387. Epub 2021 Apr 2. PMID: 33848921
Free PMC article. No abstract available.

Do gene-environment interactions play a role in COVID-19 distribution? The case of Alpha-1 Antitrypsin, air pollution and COVID-19.
Murgia N, Corsico AG, D’Amato G, Maesano CN, Tozzi A, Annesi-Maesano I. Multidiscip Respir Med. 2021 Apr 22;16(1):741. doi: 10.4081/mrm.2021.741. eCollection 2021 Jan 15.
PMID: 34012547 Free PMC article.

March 2021 Publications

Alpha-1-antitrypsin: A possible host protective factor against Covid-19.
de Loyola MB, Dos Reis TTA, de Oliveira GXLM, da Fonseca Palmeira J, Argañaraz GA, Argañaraz ER.
Rev Med Virol. 2021 Mar;31(2):e2157. doi: 10.1002/rmv.2157. Epub 2020 Aug 26. PMID: 32844538 Free PMC article. Review.

Plasma Cytokeratin 18 and fecal Alpha-1 Antitrypsin concentrations in dogs with osteosarcoma receiving carboplatin chemotherapy.
Taikowski K, Rudinsky AJ, Louke DS, Warry E, Fenger JM.
Vet Med Sci. 2021 Mar;7(2):385-392. doi: 10.1002/vms3.392. Epub 2020 Nov 22. PMID: 33222415

SZ alpha-1 antitrypsin deficiency and pulmonary disease: more like MZ, not like ZZ. Franciosi AN, Carroll TP, McElvaney NG.
Thorax. 2021 Mar;76(3):298-301. doi: 10.1136/thoraxjnl-2020-215250. Epub 2020 Sep 11.
PMID: 32917839

Alpha-1 antitrypsin governs alcohol-related liver disease in mice and humans.
Grander C, Schaefer B, Schwärzler J, Grabherr F, de Graaf DM, Enrich B, Oberhuber G, Mayr L, Sangineto M, Jaschke N, Adolph TE, Effenberger M, Moschen AR, Dinarello CA, Zoller H, Tilg H. Gut. 2021 Mar;70(3):585-594. doi: 10.1136/gutjnl-2020-321375. Epub 2020 Jul 22.
PMID: 32699098

Serpins, New Therapeutic Targets for Hemophilia.
Aymonnier K, Kawecki C, Arocas V, Boulaftali Y, Bouton MC.
Thromb Haemost. 2021 Mar;121(3):261-269. doi: 10.1055/s-0040-1716751. Epub 2020 Sep 28. PMID: 32987444

Human plasma-derived alpha1 -proteinase inhibitor in patients with new-onset type 1 diabetes mellitus: A randomized, placebo-controlled proof-of-concept study.
Lagarde WH, Courtney KL, Reiner B, Steinmann K, Tsalikian E, Willi SM.
Pediatr Diabetes. 2021 Mar;22(2):192-201. doi: 10.1111/pedi.13162. Epub 2020 Dec 13. PMID: 33244872 Free PMC article.

Alpha-1 antitrypsin inhibits TMPRSS2 protease activity and SARS-CoV-2 infection. Wettstein L, Weil T, Conzelmann C, Müller JA, Groß R, Hirschenberger M, Seidel A, Klute S,
Zech F, Prelli Bozzo C, Preising N, Fois G, Lochbaum R, Knaff PM, Mailänder V, Ständker L, Thal DR, Schumann C, Stenger S, Kleger A, Lochnit G, Mayer B, Ruiz-Blanco YB, Hoffmann M, Sparrer KMJ, Pöhlmann S, Sanchez-Garcia E, Kirchhoff F, Frick M, Münch J.
Nat Commun. 2021 Mar 19;12(1):1726. doi: 10.1038/s41467-021-21972-0. PMID: 33741941

Thrombin-like serine protease, antiquorin from Euphorbia antiquorum latex induces platelet aggregation via PAR1-Akt/p38 signaling axis.
Urs AP, Manjuprasanna VN, Rudresha GV, Hiremath V, Sharanappa P, Rajaiah R, Vishwanath BS. Biochim Biophys Acta Mol Cell Res. 2021 Mar;1868(3):118925. doi: 10.1016/j.bbamcr.2020.118925. Epub 2020 Dec 14.
PMID: 33333088

Severe alpha-1-antitrypsin deficiency increases the risk of venous thromboembolism. Basil N, Ekström M, Piitulainen E, Lindberg A, Rönmark E, Jehpsson L, Tanash H. J Thromb Haemost. 2021 Mar 24. doi: 10.1111/jth.15302. Online ahead of print.
PMID: 33763945

Known Mutations at the Cause of Alpha-1 Antitrypsin Deficiency an Updated Overview of SERPINA1 Variation Spectrum.
Seixas S, Marques PI. Appl Clin Genet. 2021 Mar 22;14:173-194. doi: 10.2147/TACG.S257511. eCollection 2021. PMID: 33790624 Free PMC article. Review.

Anti-dengue infectivity evaluation of bioflavonoid from Azadirachta indica by dengue virus serine protease inhibition.
Dwivedi VD, Bharadwaj S, Afroz S, Khan N, Ansari MA, Yadava U, Tripathi RC, Tripathi IP, Mishra SK, Kang SG.
J Biomol Struct Dyn. 2021 Mar;39(4):1417-1430. doi: 10.1080/07391102.2020.1734485. Epub 2020 Mar 9.
PMID: 32107969

The effects of non-surgical periodontal therapy on neutrophil elastase and elastase alpha-1 proteinase inhibitor levels in GCF in periodontitis patients with or without acute coronary syndrome.
Kayar NA, Üstün K, Gözlü M, Haliloğlu S, Alptekin NÖ.
Clin Oral Investig. 2021 Mar 9. doi: 10.1007/s00784-021-03838-3. Online ahead of print. PMID: 33687554

Effect of α1 antitrypsin deficiency on lung volume decline in severe asthmatic patients undergoing biologic therapy.
Vianello A, Caminati M, Senna G, Arcolaci A, Chieco-Bianchi F, Ferrarotti I, Guarnieri G, Molena B, Crisafulli E.
J Allergy Clin Immunol Pract. 2021 Mar;9(3):1414-1416. doi: 10.1016/j.jaip.2020.12.061. Epub 2021 Jan 10.
PMID: 33440256 No abstract available.

Impact of serine protease inhibitor alpha1-antitrypsin on expression of endoplasmic reticulum stress-induced proinflammatory factors in adipocytes.
Ando Y, Kuroda A, Kusama K, Matsutani T, Matsuda A, Tamura K.
Biochem Biophys Rep. 2021 Mar 2;26:100967. doi: 10.1016/j.bbrep.2021.100967. eCollection 2021 Jul.
PMID: 33732904 Free PMC article.

Alpha-1 antitrypsin (AAT) augmentation therapy in individuals with the PI*MZ genotype: a pro/con debate on a working hypothesis.
Barjaktarevic I, Miravitlles M.
BMC Pulm Med. 2021 Mar 23;21(1):99. doi: 10.1186/s12890-021-01466-x.
PMID: 33757485 Free PMC article. Review.

There is No Fast Track to Identify Fast Decliners in Alpha-1 Antitrypsin Deficiency by Spirometry: A Longitudinal Study of Repeated Measurements.
Stockley JA, Stockley RA, Sapey E. Int J Chron Obstruct Pulmon Dis. 2021 Mar 29;16:835-840. doi: 10.2147/COPD.S298585. eCollection 2021.
PMID: 33824583 Free PMC article.

Nephrotic syndrome secondary to alpha-1 antitrypsin deficiency. Santos GF, Ellis P, Farrugia D, Turner AM.
BMJ Case Rep. 2021 Mar 5;14(3):e240288. doi: 10.1136/bcr-2020-240288. PMID: 33674298

Development of a small molecule that corrects misfolding and increases secretion of Z α1 – antitrypsin.
Lomas DA, Irving JA, Arico-Muendel C, Belyanskaya S, Brewster A, Brown M, Chung CW, Dave H, Denis A, Dodic N, Dossang A, Eddershaw P, Klimaszewska D, Haq I, Holmes DS, Hutchinson JP, Jagger AM, Jakhria T, Jigorel E, Liddle J, Lind K, Marciniak SJ, Messer J, Neu M, Olszewski A, Ordonez A, Ronzoni R, Rowedder J, Rüdiger M, Skinner S, Smith KJ, Terry R, Trottet L, Uings I, Wilson S, Zhu Z, Pearce AC. EMBO Mol Med. 2021 Mar 5;13(3):e13167. doi: 10.15252/emmm.202013167. Epub 2021 Jan 29. PMID: 33512066 Free PMC article.

PNPLA3 and SERPINA1 Variants Are Associated with Severity of Fatty Liver Disease at First Referral to a Tertiary Center.
Semmler G, Balcar L, Oberkofler H, Zandanell S, Strasser M, Niederseer D, Feldman A, Stickel F, Strnad P, Datz C, Paulweber B, Aigner E.
J Pers Med. 2021 Mar 1;11(3):165. doi: 10.3390/jpm11030165.
PMID: 33804385 Free PMC article.

Intrahepatic cholangiocarcinoma in a non-cirrhotic liver in a patient with homozygous ZZ alpha-1 antitrypsin deficiency.
Vuurberg NE, Van den Boom AL, Van den Heuvel MC, Klaase JM.
BMJ Case Rep. 2021 Mar 3;14(3):e240077. doi: 10.1136/bcr-2020-240077.
PMID: 33658218 Free PMC article.

The development of highly potent and selective small molecule correctors of Z α1-antitrypsin misfolding.
Liddle J, Pearce AC, Arico-Muendel C, Belyanskaya S, Brewster A, Brown M, Chung CW, Denis A, Dodic N, Dossang A, Eddershaw P, Klimaszewska D, Haq I, Holmes DS, Jagger A, Jakhria T, Jigorel E, Lind K, Messer J, Neu M, Olszewski A, Ronzoni R, Rowedder J, Rüdiger M, Skinner S, Smith KJ, Trottet L, Uings I, Zhu Z, Irving JA, Lomas DA.
Bioorg Med Chem Lett. 2021 Mar 19;41:127973. doi: 10.1016/j.bmcl.2021.127973. Online ahead of print.
PMID: 33753261

Renal effects of the serine protease inhibitor aprotinin in healthy conscious mice.
Wörner S, Bohnert BN, Wörn M, Xiao M, Janessa A, Birkenfeld AL, Amann K, Daniel C, Artunc F.
Acta Pharmacol Sin. 2021 Mar 23. doi: 10.1038/s41401-021-00628-1. Online ahead of print. PMID: 33758357

Identification of an alpha-1 antitrypsin variant with enhanced specificity for factor XIa by phage display, bacterial expression, and combinatorial mutagenesis.
Bhakta V, Hamada M, Nouanesengsy A, Lapierre J, Perruzza DL, Sheffield WP. Sci Rep. 2021 Mar 10;11(1):5565. doi: 10.1038/s41598-021-84618-7.
PMID: 33692375 Free PMC article.

Serine protease inhibitors of the whirling disease parasite Myxobolus cerebralis (Cnidaria, Myxozoa): Expression profiling and functional predictions.
Eszterbauer E, Szegő D, Ursu K, Sipos D, Gellért Á.
PLoS One. 2021 Mar 29;16(3):e0249266. doi: 10.1371/journal.pone.0249266. eCollection 2021. PMID: 33780500

Small airway evaluation in three subjects with alpha-1 antitrypsin deficiency without diagnosed lung disease.
Ferrari da Cruz T, Rufino R, Lopes A, Henrique Costa C.
BMJ Case Rep. 2021 Mar 30;14(3):e239146. doi: 10.1136/bcr-2020-239146. PMID: 33785603

Carbohydrate-controlled serine protease inhibitor (serpin) production in Bifidobacterium longum subsp. longum.
Duboux S, Golliard M, Muller JA, Bergonzelli G, Bolten CJ, Mercenier A, Kleerebezem M. Sci Rep. 2021 Mar 31;11(1):7236. doi: 10.1038/s41598-021-86740-y.
PMID: 33790385 Free PMC article.

High level expression and biochemical characterization of an alkaline serine protease from Geobacillus stearothermophilus to prepare antihypertensive whey protein hydrolysate.
Chang C, Gong S, Liu Z, Yan Q, Jiang Z.
BMC Biotechnol. 2021 Mar 11;21(1):21. doi: 10.1186/s12896-021-00678-7.
PMID: 33706728 Free PMC article.

Safety and efficacy of a feed additive consisting of serine protease produced by Bacillus licheniformisDSM 19670 (Ronozyme^® ProAct) for chickens for fattening (DSM Nutritional Products Ltd.).
EFSA Panel on Additives and Products or Substances used in Animal Feed (FEEDAP), Bampidis V, Azimonti G, Bastos ML, Christensen H, Dusemund B, Kouba M, Durjava MF, López-Alonso M, López Puente S, Marcon F, Mayo B, Pechová A, Petkova M, Ramos F, Sanz Y, Villa RE, Cocconcelli PS, Glandorf B, Herman L, Maradona MP, Saarela M, Anguita M, Galobart J, Holczknecht O, Manini P, Pizzo F, Tarrés-Call J, Pettenati E.
EFSA J. 2021 Mar 5;19(3):e06448. doi: 10.2903/j.efsa.2021.6448. eCollection 2021 Mar. PMID: 33727967 Free PMC article.

The levels of the serine protease HTRA1 in cerebrospinal fluid correlate with progression and disability in multiple sclerosis.
Hjæresen S, Sejbaek T, Axelsson M, Vinsløv-Jensen H, Mortensen SK, Pihl-Jensen G, Novakova L, Christensen JDR, Pedersen CB, Halle B, Poulsen FR, Lautrup Frederiksen J, Zhang M, Benedikz E, Lycke J, Illes Z, Fex Svenningsen Å.
J Neurol. 2021 Mar 4. doi: 10.1007/s00415-021-10489-7. Online ahead of print. PMID: 33661357

Oncogenic Ras Disrupts Epithelial Integrity by Activating the Transmembrane Serine Protease Hepsin.
Tervonen TA, Pant SM, Belitškin D, Englund JI, Närhi K, Haglund C, Kovanen PE, Verschuren EW, Klefström J.
Cancer Res. 2021 Mar 15;81(6):1513-1527. doi: 10.1158/0008-5472.CAN-20-1760. Epub 2021 Jan 18.
PMID: 33461973

Lint, a transmembrane serine protease, regulates growth and metabolism in Drosophila. Pathak H, Maya AV, Tanari AB, Sarkar S, Varghese J.
Genetics. 2021 Mar 9:iyab035. doi: 10.1093/genetics/iyab035. Online ahead of print. PMID: 33693655

A Particular SORL1 Micro-Haplotype May Prevent from Severe Liver Disease in a French Cohort of Alpha 1-Antitrypsin Deficiency Children.
Joly P, Ruiz M, Garin R, Karatas E, Lachaux A, Restier L, Belmalih A, Renoux C, Lombard C, Dechomet M, Bouchecareilh M.
J Pediatr Gastroenterol Nutr. 2021 Mar 12. doi: 10.1097/MPG.0000000000003125. Online ahead of print.
PMID: 33720088

In-silico screening for identification of potential inhibitors against SARS-CoV-2 transmembrane serine protease 2 (TMPRSS2).
Barge S, Jade D, Gosavi G, Talukdar NC, Borah J.
Eur J Pharm Sci. 2021 Mar 26;162:105820. doi: 10.1016/j.ejps.2021.105820. Online ahead of print. PMID: 33775827 Free PMC article.

Magnesium Treatment on Methylation Changes of Transmembrane Serine Protease 2 (TMPRSS2). Fan L, Zhu X, Zheng Y, Zhang W, Seidner DL, Ness R, Murff HJ, Yu C, Huang X, Shrubsole MJ, Hou L, Dai Q. medRxiv. 2021 Mar 12:2021.03.11.21253287. doi: 10.1101/2021.03.11.21253287. Preprint. PMID: 33758885 Free PMC article.

The Anti-Inflammatory Immune Response in Early Trichinella spiralis Intestinal Infection Depends on Serine Protease Inhibitor-Mediated Alternative Activation of Macrophages.
Xu N, Bai X, Liu Y, Yang Y, Tang B, Shi HN, Vallee I, Boireau P, Liu X, Liu M.
J Immunol. 2021 Mar 1;206(5):963-977. doi: 10.4049/jimmunol.2000290. Epub 2021 Jan 25. PMID: 33495238 Free PMC article.

Elucidating the role of GRIM-19 as a substrate and allosteric activator of pro-apoptotic serine protease HtrA2.
Kummari R, Dutta S, Patil S, Mudrale SP, Bose K.
Biochem J. 2021 Mar 26;478(6):1241-1259. doi: 10.1042/BCJ20200923. PMID: 33650635

Efficient Hydrolysis of Gluten-Derived Celiac Disease-Triggering Immunogenic Peptides by a Bacterial Serine Protease from Burkholderia gladioli.
Liu YY, Lee CC, Hsu JH, Leu WM, Meng M.
Biomolecules. 2021 Mar 17;11(3):451. doi: 10.3390/biom11030451. PMID: 33802942 Free PMC article.

Disruption of Epithelial Barrier of Caco-2 Cell Monolayers by Excretory Secretory Products of Trichinella spiralis Might Be Related to Serine Protease.
Li C, Bai X, Liu X, Zhang Y, Liu L, Zhang L, Xu F, Yang Y, Liu M. Front Microbiol. 2021 Mar 17;12:634185. doi: 10.3389/fmicb.2021.634185. eCollection 2021. PMID: 33815318 Free PMC article.

February 2021 Publications

Small-molecule modulators of serine protease inhibitor proteins (serpins). Kellici TF, Pilka ES, Bodkin MJ.
Drug Discov Today. 2021 Feb;26(2):442-454. doi: 10.1016/j.drudis.2020.11.012. Epub 2020 Nov 28.
PMID: 33259801 Review.

Alpha-1 antitrypsin deficiency and recombinant protein sources with focus on plant sources: Updates, challenges and perspectives.
McNulty MJ, Silberstein DZ, Kuhn BT, Padgett HS, Nandi S, McDonald KA, Cross CE.
Free Radic Biol Med. 2021 Feb 1;163:10-30. doi: 10.1016/j.freeradbiomed.2020.11.030. Epub 2020 Dec 3.
PMID: 33279618 Review.

A critical review on marine serine protease and its inhibitors: A new wave of drugs?
Barzkar N, Khan Z, Tamadoni Jahromi S, Pourmozaffar S, Gozari M, Nahavandi R.
Int J Biol Macromol. 2021 Feb 15;170:674-687. doi: 10.1016/j.ijbiomac.2020.12.134. Epub 2020 Dec 30.
PMID: 33387547 Review.

Overexpression of alpha-1 antitrypsin in mesenchymal stromal cells improves their intrinsic biological properties and therapeutic effects in nonobese diabetic mice.
Song L, Gou W, Wang J, Wei H, Lee J, Strange C, Wang H.
Stem Cells Transl Med. 2021 Feb;10(2):320-331. doi: 10.1002/sctm.20-0122. Epub 2020 Sep 18. PMID: 32945622 Free PMC article.

Homology modeling and heterologous expression of highly alkaline subtilisin-like serine protease from Bacillus halodurans C-125.
Tekin A, Uzuner U, Sezen K.
Biotechnol Lett. 2021 Feb;43(2):479-494. doi: 10.1007/s10529-020-03025-6. Epub 2020 Oct 12. PMID: 33047274

Human eggs, zygotes, and embryos express the receptor angiotensin 1-converting enzyme 2 and transmembrane serine protease 2 protein necessary for severe acute respiratory syndrome coronavirus 2 infection.
Rajput SK, Logsdon DM, Kile B, Engelhorn HJ, Goheen B, Khan S, Swain J, McCormick S, Schoolcraft WB, Yuan Y, Krisher RL.
F S Sci. 2021 Feb;2(1):33-42. doi: 10.1016/j.xfss.2020.12.005. Epub 2020 Dec 24.
PMID: 33521687 Free PMC article.

Reduced 25(OH) Vitamin D Association with Lower Alpha-1-Antitrypsin Blood Levels in Type 2 Diabetic Patients.
Lindley VM, Bhusal K, Huning L, Levine SN, Jain SK.
J Am Coll Nutr. 2021 Feb;40(2):98-103. doi: 10.1080/07315724.2020.1740629. Epub 2020 Apr 10. PMID: 32275481

Structural modelling and thermostability of a serine protease inhibitor belonging to the Kunitz-BPTI family from the Rhipicephalus microplus tick.
Bomediano Camillo LM, Ferreira GC, Duran AFA, da Silva FRS, Garcia W, Scott AL, Sasaki SD. Biochimie. 2021 Feb;181:226-233. doi: 10.1016/j.biochi.2020.12.014. Epub 2020 Dec 24.
PMID: 33359560

Two steps purification, biochemical characterization, thermodynamics and structure elucidation of thermostable alkaline serine protease from Nocardiopsis alba strain OM-5.
Chauhan JV, Mathukiya RP, Singh SP, Gohel SD.
Int J Biol Macromol. 2021 Feb 1;169:39-50. doi: 10.1016/j.ijbiomac.2020.12.061. Epub 2020 Dec 11.

Identification of creatine kinase and alpha-1 antitrypsin as protein targets of alkylation by sulfur mustard.
Lüling R, Schmeißer W, Siegert M, Mückter H, Dietrich A, Thiermann H, Gudermann T, John H, Steinritz D.
Drug Test Anal. 2021 Feb;13(2):268-282. doi: 10.1002/dta.2916. Epub 2020 Sep 18. PMID: 32852113

Alpha-1 Antitrypsin Deficiency and Tobacco Smoking: Exploring Risk Factors and Smoking Cessation in a Registry Population.
Franciosi AN, Alkhunaizi MA, Woodsmith A, Aldaihani L, Alkandari H, Lee SE, Fee LT, McElvaney NG, Carroll TP.
COPD. 2021 Feb;18(1):76-82. doi: 10.1080/15412555.2020.1864725. Epub 2021 Feb 9.
PMID: 33557645

Alpha-1 antitrypsin deficiency impairs lung antibacterial immunity in mice.
Ostermann L, Maus R, Stolper J, Schütte L, Katsarou K, Tumpara S, Pich A, Mueller C, Janciauskiene S, Welte T, Maus UA.
JCI Insight. 2021 Feb 8;6(3):e140816. doi: 10.1172/jci.insight.140816. PMID: 33554955 Free PMC article.

A general strategy to inhibit serine protease by targeting its autolysis loop. Jiang L, Yuan C, Huang M.
FASEB J. 2021 Feb;35(2):e21259. doi: 10.1096/fj.202002139RR. PMID: 33417271 Review.

Host genomics of COVID-19: Evidence point towards Alpha 1 antitrypsin deficiency as a putative risk factor for higher mortality rate.
Dutta AK, Goswami K.
Med Hypotheses. 2021 Feb;147:110485. doi: 10.1016/j.mehy.2021.110485. Epub 2021 Jan 9. PMID: 33450625 Free PMC article.

Origin and Expansion of the Serine Protease Repertoire in the Myelomonocyte Lineage. Weiss SAI, Rehm SRT, Perera NC, Biniossek ML, Schilling O, Jenne DE.
Int J Mol Sci. 2021 Feb 7;22(4):1658. doi: 10.3390/ijms22041658.
PMID: 33562184 Free PMC article. Review.

Clarifying the relationship between pulmonary langerhans cell histiocytosis and Alpha 1 antitrypsin deficiency.
McCarthy C, Bugnet E, Benattia A, Keane MP, Vedie B, Lorillon G, Tazi A. Orphanet J Rare Dis. 2021 Feb 9;16(1):72. doi: 10.1186/s13023-021-01720-9.
PMID: 33563302 Free PMC article.

Hepatobiliary phenotypes of adults with alpha-1 antitrypsin deficiency.
Fromme M, Schneider CV, Pereira V, Hamesch K, Pons M, Reichert MC, Benini F, Ellis P, H Thorhauge K, Mandorfer M, Burbaum B, Woditsch V, Chorostowska-Wynimko J, Verbeek J, Nevens F, Genesca J, Miravitlles M, Nuñez A, Schaefer B, Zoller H, Janciauskiene S, Abreu N, Jasmins L, Gaspar R, Gomes C, Schneider KM, Trauner M, Krag A, Gooptu B, Thorburn D, Marshall A, Hurst JR, Lomas DA, Lammert F, Gaisa NT, Clark V, Griffiths W, Trautwein C, Turner AM, McElvaney NG, Strnad P.
Gut. 2021 Feb 25:gutjnl-2020-323729. doi: 10.1136/gutjnl-2020-323729. Online ahead of print. PMID: 33632708

The Autophagy Pathway: A Critical Route in the Disposal of Alpha 1-Antitrypsin Aggregates That Holds Many Mysteries.
Leon C, Bouchecareilh M.
Int J Mol Sci. 2021 Feb 13;22(4):1875. doi: 10.3390/ijms22041875.
PMID: 33668611 Free PMC article. Review.

Prevalence and clinical characteristics of alpha-1 antitrypsin deficiency in liver explants in a Mexican cohort.
Campos-Murguía A, Valdéz-Hernández P, Cordova-Gallardo J, Arteaga-Vázquez J, Contreras AG, Vilatobá M, Cruz-Martínez R, Martínez-Benítez B, Gamboa-Domínguez A, Marfil-Garza BA, Flores-García NC, Márquez-Guillén E, García-Juárez I. Clin Res Hepatol Gastroenterol. 2021 Feb 23;45(4):101519. doi: 10.1016/j.clinre.2020.07.024. Online ahead of print.
PMID: 33636655

Knockdown of Alpha-1 Antitrypsin with antisense oligonucleotide does not exacerbate smoke induced lung injury.
Stearns K, Goldklang M, Xiao R, Zelonina T, Blomenkamp K, Teckman J, D’Armiento JM. PLoS One. 2021 Feb 4;16(2):e0246040. doi: 10.1371/journal.pone.0246040. eCollection 2021.
PMID: 33539438 Free PMC article.

Dutomycin Induces Autophagy and Apoptosis by Targeting the Serine Protease Inhibitor SERPINB6.
Jang M, Hara S, Kim GH, Kim SM, Son S, Kwon M, Ryoo IJ, Seo H, Kim MJ, Kim ND, Soung NK, Kwon YT, Kim BY, Osada H, Jang JH, Ko SK, Ahn JS.
ACS Chem Biol. 2021 Feb 19;16(2):360-370. doi: 10.1021/acschembio.0c00889. Epub 2021 Jan 31.
PMID: 33517652

Air Trapping Is Associated with Heterozygosity for Alpha-1 Antitrypsin Mutations in Patients with Asthma.
Aiello M, Ghirardini M, Marchi L, Frizzelli A, Pisi R, Ferrarotti I, Bertorelli G, Chetta A. Respiration. 2021 Feb 5:1-10. doi: 10.1159/000513072. Online ahead of print.
PMID: 33550308

A new electrochemical impedance biosensor based on aromatic thiol for alpha-1 antitrypsin determination.
YaĞar H, Özcan HM, Mehmet O. Turk J Chem. 2021 Feb 17;45(1):104-118. doi: 10.3906/kim-2007-6. eCollection 2021. PMID: 33679157 Free PMC article.

A Novel Mouse Monoclonal Antibody C42 against C-Terminal Peptide of Alpha-1-Antitrypsin. Tumpara S, Korenbaum E, Kühnel M, Jonigk D, Olejnicka B, Davids M, Welte T, Martinez- Delgado B, Janciauskiene S. Int J Mol Sci. 2021 Feb 21;22(4):2141. doi: 10.3390/ijms22042141. PMID: 33670003 Free PMC article.

TKP, a Serine Protease from Trichosanthes kirilowii, Inhibits Cell Proliferation by Blocking Aerobic Glycolysis in Hepatocellular Carcinoma Cells.
Zhao H, Song L. Nutr Cancer. 2021 Feb 5:1-13. doi: 10.1080/01635581.2021.1882508. Online ahead of print. PMID: 33544002

Insight into the collagen-degrading activity of a serine protease in the latex of Ficus carica cultivar Masui Dauphine.
Nishimura K, Higashiya K, Ueshima N, Kojima K, Takita T, Abe T, Takahashi T, Yasukawa K. Biosci Biotechnol Biochem. 2021 Feb 13:zbab025. doi: 10.1093/bbb/zbab025. Online ahead of print.
PMID: 33580958

Targeting the Complement Serine Protease MASP-2 as a Therapeutic Strategy for Coronavirus Infections.
Flude BM, Nannetti G, Mitchell P, Compton N, Richards C, Heurich M, Brancale A, Ferla S, Bassetto M.
Viruses. 2021 Feb 17;13(2):312. doi: 10.3390/v13020312.
PMID: 33671334 Free PMC article.

Janurary 2021 Publications

Hypothesis: Alpha-1-antitrypsin is a promising treatment option for COVID-19.
Bai X, Hippensteel J, Leavitt A, Maloney JP, Beckham D, Garcia C, Li Q, Freed BM, Ordway D, Sandhaus RA, Chan ED.
Med Hypotheses. 2021 Jan;146:110394. doi: 10.1016/j.mehy.2020.110394. Epub 2020 Nov 12. PMID: 33239231 Free PMC article. Review.

Alpha 1 Antitrypsin Deficiency.
Meseeha M, Attia M.
2020 Aug 10. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan–. PMID: 28723059 Free Books & Documents. Review.

Alpha 1 Antitrypsin Mutation. Abdulkarim A, Craig TJ.
2020 Jul 15. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan–. PMID: 29489299 Free Books & Documents. Review.

Alpha-1 antitrypsin deficiency and risk of lung cancer: A systematic review. Tubío-Pérez RA, Torres-Durán M, Fernández-Villar A, Ruano-Raviña A.
Transl Oncol. 2021 Jan;14(1):100914. doi: 10.1016/j.tranon.2020.100914. Epub 2020 Nov 1. PMID: 33142121 Free PMC article.

Alpha-1 Antitrypsin Deficiency and COVID-19 Infection. Strassmair M, Stangl M.
Arch Bronconeumol. 2021 Jan;57 Suppl 1:97. doi: 10.1016/j.arbres.2020.10.009. Epub 2020 Oct 22.
PMID: 33189418 Free PMC article. No abstract available.

An integrative view of serpins in health and disease: the contribution of SerpinA3. Sánchez-Navarro A, González-Soria I, Caldiño-Bohn R, Bobadilla NA.
Am J Physiol Cell Physiol. 2021 Jan 1;320(1):C106-C118. doi: 10.1152/ajpcell.00366.2020. Epub 2020 Oct 28.
PMID: 33112643

Alpha-1-antitrypsin deficiency (AATD) and spontaneous pneumothorax: Guidelines do not recommend screening for AATD in patients with pneumothorax – What did we find in 10 years of clinical evidence? Dias E, Farinha I, Costa F.
Pulmonology. 2021 Jan-Feb;27(1):80-81. doi: 10.1016/j.pulmoe.2020.05.014. Epub 2020 Jun 14. PMID: 32553825 No abstract available.

Alpha-1 antitrypsin for cystic fibrosis complicated by severe cytokinemic COVID-19.
McElvaney OJ, O’Connor E, McEvoy NL, Fraughan DD, Clarke J, McElvaney OF, Gunaratnam C, O’Rourke J, Curley GF, McElvaney NG.
J Cyst Fibros. 2021 Jan;20(1):31-35. doi: 10.1016/j.jcf.2020.11.012. Epub 2020 Nov 20. PMID: 33288475 Free PMC article.

A novel serine protease with anticoagulant and fibrinolytic activities from the fruiting bodies of mushroom Agrocybe aegerita.
Li G, Liu X, Cong S, Deng Y, Zheng X.
Int J Biol Macromol. 2021 Jan 31;168:631-639. doi: 10.1016/j.ijbiomac.2020.11.118. Epub 2020 Nov 20.
PMID: 33227332

Alpha-1 antitrypsin deficiency-associated panniculitis.
Franciosi AN, Ralph J, O’Farrell NJ, Buckley C, Gulmann C, O’Kane M, Carroll TP, McElvaney NG.
J Am Acad Dermatol. 2021 Jan 29:S0190-9622(21)00232-2. doi: 10.1016/j.jaad.2021.01.074.
Online ahead of print. PMID: 33516773 Review.

Exacerbations of Lung Disease in Alpha-1 Antitrypsin Deficiency. Smith DJ, Ellis PR, Turner AM.
Chronic Obstr Pulm Dis. 2021 Jan;8(1). doi: 10.15326/jcopdf.2020.0173. PMID: 33238089

Serpins in Hemostasis as Therapeutic Targets for Bleeding or Thrombotic Disorders. Bianchini EP, Auditeau C, Razanakolona M, Vasse M, Borgel D.
Front Cardiovasc Med. 2021 Jan 7;7:622778. doi: 10.3389/fcvm.2020.622778. eCollection 2020. PMID: 33490121 Free PMC article. Review.

Deriving Immune-Modulating Peptides from Viral Serine Protease Inhibitors (Serpins).
Yaron JR, Zhang L, Burgin M, Schutz LN, Awo EA, Keinan S, McFadden G, Ambadapadi S, Guo Q, Chen H, Lucas AR.
Methods Mol Biol. 2021;2225:107-123. doi: 10.1007/978-1-0716-1012-1_7. PMID: 33108660

ACE2 (Angiotensin-Converting Enzyme 2) and TMPRSS2 (Transmembrane Serine Protease 2) Expression and Localization of SARS-CoV-2 Infection in the Human Heart.
Sakamoto A, Kawakami R, Kawai K, Gianatti A, Pellegrini D, Kutys R, Guo L, Mori M, Cornelissen A, Sato Y, Bellasi A, Faggi L, Hong C, Romero M, Guagliumi G, Virmani R, Finn AV. Arterioscler Thromb Vasc Biol. 2021 Jan;41(1):542-544. doi: 10.1161/ATVBAHA.120.315229.
Epub 2020 Oct 22.
PMID: 33086866 Review. No abstract available.

Protease-Specific Biomarkers to Analyse Protease Inhibitors for Emphysema Associated with Alpha 1-Antitrypsin Deficiency. An Overview of Current Approaches.
Viglio S, Bak EG, Schouten IGM, Iadarola P, Stolk J.
Int J Mol Sci. 2021 Jan 21;22(3):1065. doi: 10.3390/ijms22031065.
PMID: 33494436 Free PMC article. Review.

SARS-CoV-2 entry into human airway organoids is serine protease-mediated and facilitated by the multibasic cleavage site.
Mykytyn AZ, Breugem TI, Riesebosch S, Schipper D, van den Doel PB, Rottier RJ, Lamers MM, Haagmans BL.
Elife. 2021 Jan 4;10:e64508. doi: 10.7554/eLife.64508. PMID: 33393462 Free PMC article.

Design, synthesis, and evaluation of Bothrops venom serine protease peptidic inhibitors.
da Silva GM, de Souza DHB, Waitman KB, Ebram MC, Fessel MR, Zainescu IC, Portaro FC, Heras M, de Andrade SA.
J Venom Anim Toxins Incl Trop Dis. 2021 Jan 15;27:e20200066. doi: 10.1590/1678-9199- JVATITD-2020-0066. eCollection 2021.
PMID: 33488681 Free PMC article.

Anticoagulant and antiplatelet activities of novel serine protease purified from seeds of Cucumis maderaspatensis.
Sachin HR, SharathKumar MN, Devaraja S, Sneharani AH.
3 Biotech. 2021 Jan;11(1):30. doi: 10.1007/s13205-020-02565-y. Epub 2021 Jan 4.
PMID: 33457164

Home-Based Multicomponent Intervention Increases Exercise Activity and Improves Body Mass Index: Results of a 5-Year Randomized Trial Among Individuals with Alpha-1 Antitrypsin Deficiency-Associated Lung Disease.
Choate R, Mannino DM, Holm KE, Beiko T, Boyd B, Sandhaus RA. Chronic Obstr Pulm Dis. 2021 Jan;8(1). doi: 10.15326/jcopdf.8.1.2020.0183.
PMID: 33135406

The Clinical Utility of Determining the Allelic Background of Mutations Causing Alpha-1 Antitrypsin Deficiency: The Case with the Null Variant Q0(Mattawa)/Q0(Ourém).
Bellemare J, Gaudreault N, Valette K, Belmonte I, Nuñez A, Miravitlles M, Maltais F, Bossé Y. Chronic Obstr Pulm Dis. 2021 Jan;8(1). doi: 10.15326/jcopdf.8.1.2020.0168.
PMID: 33150777

Development of a small molecule that corrects misfolding and increases secretion of Z α1 – antitrypsin.
Lomas DA, Irving JA, Arico-Muendel C, Belyanskaya S, Brewster A, Brown M, Chung CW, Dave H, Denis A, Dodic N, Dossang A, Eddershaw P, Klimaszewska D, Haq I, Holmes DS, Hutchinson JP, Jagger AM, Jakhria T, Jigorel E, Liddle J, Lind K, Marciniak SJ, Messer J, Neu M, Olszewski A, Ordonez A, Ronzoni R, Rowedder J, Rüdiger M, Skinner S, Smith KJ, Terry R, Trottet L, Uings I, Wilson S, Zhu Z, Pearce AC.
EMBO Mol Med. 2021 Mar 5;13(3):e13167. doi: 10.15252/emmm.202013167. Epub 2021 Jan 29.
PMID: 33512066 Free PMC article.

Variation in the SERPINA6/SERPINA1 locus alters morning plasma cortisol, hepatic corticosteroid binding globulin expression, gene expression in peripheral tissues, and risk of cardiovascular disease.
Crawford AA, Bankier S, Altmaier E, Barnes CLK, Clark DW, Ermel R, Friedrich N, van der Harst P, Joshi PK, Karhunen V, Lahti J, Mahajan A, Mangino M, Nethander M, Neumann A, Pietzner M, Sukhavasi K, Wang CA, Bakker SJL, Bjorkegren JLM, Campbell H, Eriksson J, Gieger C, Hayward C, Jarvelin MR, McLachlan S, Morris AP, Ohlsson C, Pennell CE, Price J, Rudan I, Ruusalepp A, Spector T, Tiemeier H, Völzke H, Wilson JF, Michoel T, Timpson NJ, Smith GD, Walker BR; CORtisol NETwork (CORNET) consortium.
J Hum Genet. 2021 Jan 20. doi: 10.1038/s10038-020-00895-6. Online ahead of print. PMID: 33469137

Consequences of ‘no-choice, fixed time’ reciprocal host plant switches on nutrition and gut serine protease gene expression in Pieris brassicae L. (Lepidoptera: Pieridae).
Kumar P, Akhter T, Bhardwaj P, Kumar R, Bhardwaj U, Mazumdar-Leighton S.
PLoS One. 2021 Jan 20;16(1):e0245649. doi: 10.1371/journal.pone.0245649. eCollection 2021. PMID: 33471847 Free PMC article.

Purification and Biochemical Characterization of a Novel Thermostable Serine Protease from Geobacillus sp. GS53.
Baykara SG, Sürmeli Y, Şanlı-Mohamed G.
Appl Biochem Biotechnol. 2021 Jan 28. doi: 10.1007/s12010-021-03512-0. Online ahead of print. PMID: 33507494

Expression Status of Serine Protease 27: A Prognostic Marker for Esophageal Squamous Cell Carcinoma Treated with Preoperative Chemotherapy/Chemoradiotherapy.
Kataoka A, Yamada K, Hagiwara T, Terayama M, Sugimoto T, Nohara K, Igari T, Yokoi C, Kawamura YI.
Ann Surg Oncol. 2021 Jan 15. doi: 10.1245/s10434-020-09550-y. Online ahead of print. PMID: 33452606

Clinical presentations of four patients with rare Alpha 1 Antitrypsin variants identified in a single US center.
Kueppers F. Respir Med Case Rep. 2021 Jan 20;32:101345. doi: 10.1016/j.rmcr.2021.101345. eCollection 2021. PMID: 33552892 Free PMC article.

ASO Author Reflections: Serine Protease 27 is a Novel Prognostic Indicator for Esophageal Squamous Cell Carcinoma with Preoperative Treatment.
Kataoka A, Yamada K, Kawamura YI. Ann Surg Oncol. 2021 Jan 16. doi: 10.1245/s10434-020-09580-6. Online ahead of print. PMID: 33454876 No abstract available.

Serendipitous detection of α1-antitrypsin deficiency: a single institution’s experience over a 32 month period.
Cronin T, Rasheed E, Naughton A, McElvaney NG, Carroll TP, Crowley VEF, Conlon N. Clin Chem Lab Med. 2021 Jan 26. doi: 10.1515/cclm-2020-1750. Online ahead of print.
PMID: 33544483 No abstract available.

A Putative Serine Protease is Required to Initiate the RIPK3-MLKL-Mediated Necroptotic Death Pathway in Neutrophils.
Wang X, Avsec D, Obreza A, Yousefi S, Mlinarič-Raščan I, Simon HU.
Front Pharmacol. 2021 Jan 21;11:614928. doi: 10.3389/fphar.2020.614928. eCollection 2020. PMID: 33551816 Free PMC article.

Liver Cirrhosis Secondary to Autoimmune Hepatitis in a Patient with Alpha-1 Antitrypsin ZZ Phenotype: A “Double Hit” Phenomenon.
Hoilat GJ, Khan A, Masood U, Sharma A, Manocha D. Cureus. 2021 Jan 10;13(1):e12606. doi: 10.7759/cureus.12606. PMID: 33585096 Free PMC article.

December 2020 Publications

Monocytes and Macrophages in Alpha-1 Antitrypsin Deficiency.
Belchamber KBR, Walker EM, Stockley RA, Sapey E.
Int J Chron Obstruct Pulmon Dis. 2020 Dec 3;15:3183-3192. doi: 10.2147/COPD.S276792. eCollection 2020.
PMID: 33311976 Free PMC article. Review.

Improving the Lives of Patients with Alpha-1 Antitrypsin Deficiency.
Sandhaus RA, Strange C, Zanichelli A, Skålvoll K, Koczulla AR, Stockley RA.
Int J Chron Obstruct Pulmon Dis. 2020 Dec 10;15:3313-3322. doi: 10.2147/COPD.S276773. eCollection 2020.
PMID: 33328731 Free PMC article. Review.

Identification of Variants in Alpha-1-Antitrypsin by High Resolution Melting.
Bidla G, Rosenblatt DS, Gilfix BM.
J Appl Lab Med. 2020 Dec 17:jfaa191. doi: 10.1093/jalm/jfaa191. Online ahead of print.
PMID: 33331634

Alpha-1 Antitrypsin for COVID-19 Treatment: Dual Role in Antiviral Infection and Anti- Inflammation.
Yang C, Keshavjee S, Liu M.
Front Pharmacol. 2020 Dec 11;11:615398. doi: 10.3389/fphar.2020.615398. eCollection 2020.
PMID: 33362565 Free PMC article.

Obstacles to Early Diagnosis and Treatment of Alpha-1 Antitrypsin Deficiency: Current Perspectives.
Quinn M, Ellis P, Pye A, Turner AM. Ther Clin Risk Manag. 2020 Dec 16;16:1243-1255. doi: 10.2147/TCRM.S234377. eCollection 2020.
PMID: 33364772 Free PMC article. Review.

The undiagnosed disease burden associated with alpha-1 antitrypsin deficiency genotypes.
Nakanishi T, Forgetta V, Handa T, Hirai T, Mooser V, Lathrop GM, Cookson WOCM, Richards JB.
Eur Respir J. 2020 Dec 10;56(6):2001441. doi: 10.1183/13993003.01441-2020. Print 2020 Dec.
PMID: 32675199 Free PMC article.

Research priorities in α1-antitrypsin deficiency: results of a patients’ and healthcare providers’ international survey from the EARCO Clinical Research Collaboration.
Barrecheguren M, O’Hara K, Wilkens M, Boyd J, Kolda E, Lara B, Chorostowska-Wynimko J, Ferrarotti I, Chlumský J, Clarenbach C, Greulich T, Miravitlles M, Sucena M.
ERJ Open Res. 2020 Dec 21;6(4):00523-2020. doi: 10.1183/23120541.00523-2020. eCollection 2020 Oct.
PMID: 33447613 Free PMC article.

Post-Translational Modifications of Circulating Alpha-1-Antitrypsin Protein.
Lechowicz U, Rudzinski S, Jezela-Stanek A, Janciauskiene S, Chorostowska-Wynimko J. Int J Mol Sci. 2020 Dec 2;21(23):9187. doi: 10.3390/ijms21239187.
PMID: 33276468 Free PMC article. Review.

PredictAAT: Accounting for Inflammation in the Diagnosis of Alpha 1 Antitrypsin Deficiency.
Kueppers F.COPD. 2020 Dec;17(6):619-622. doi: 10.1080/15412555.2020.1812055. Epub 2020 Aug 30.
PMID: 32862719

Improving Screening for Alpha-1 Antitrypsin Deficiency With Direct Testing in the Pulmonary Function Testing Laboratory.
Gurevich S, Daya A, Da Silva C, Girard C, Rahaghi F.
Chronic Obstr Pulm Dis. 2020 Dec 2. doi: 10.15326/jcopdf.2020.0179. Online ahead of print.
PMID: 33290644

Non-invasive testing for liver pathology in alpha-1 antitrypsin deficiency.
Abbas SH, Pickett E, Lomas DA, Thorburn D, Gooptu B, Hurst JR, Marshall A.
BMJ Open Respir Res. 2020 Dec;7(1):e000820. doi: 10.1136/bmjresp-2020-000820.
PMID: 33323365 Free PMC article.

Calreticulin enhances the secretory trafficking of a misfolded α-1-antitrypsin.
Mohan HM, Yang B, Dean NA, Raghavan M. J Biol Chem. 2020 Dec 4;295(49):16754-16772. doi: 10.1074/jbc.RA120.014372. PMID: 33453909

High-resolution ex vivo NMR spectroscopy of human Z α1-antitrypsin.
Jagger AM, Waudby CA, Irving JA, Christodoulou J, Lomas DA. Nat Commun. 2020 Dec 11;11(1):6371. doi: 10.1038/s41467-020-20147-7.
PMID: 33311470 Free PMC article.

Gene coexpression networks reveal novel molecular endotypes in alpha-1 antitrypsin deficiency.
Chu JH, Zang W, Vukmirovic M, Yan X, Adams T, DeIuliis G, Hu B, Mihaljinec A, Schupp JC, Becich MJ, Hochheiser H, Gibson KF, Chen ES, Morris A, Leader JK, Wisniewski SR, Zhang Y, Sciurba FC, Collman RG, Sandhaus R, Herzog EL, Patterson KC, Sauler M, Strange C, Kaminski N; GRADS Investigators.
Thorax. 2020 Dec 10:thoraxjnl-2019-214301. doi: 10.1136/thoraxjnl-2019-214301. Online ahead of print.
PMID: 33303696

Longitudinal Outcomes in Young Patients with Alpha-1-Antitrypsin Deficiency with Native Liver Reveal that Neonatal Cholestasis is a Poor Predictor of Future Portal Hypertension.
Teckman J, Rosenthal P, Hawthorne K, Spino C, Bass LM, Murray KF, Kerkar N, Magee JC, Karpen S, Heubi JE, Molleston JP, Squires RH, Kamath BM, Guthery SL, Loomes KM, Sherker AH, Sokol RJ; ChiLDReN (Childhood Liver Disease Research Network).
J Pediatr. 2020 Dec;227:81-86.e4. doi: 10.1016/j.jpeds.2020.07.031. Epub 2020 Jul 11.
PMID: 32663593

Limitations and Challenges of Conducting Budget Impact Analyses in Rare Diseases: A Case Study of Alpha-1 Antitrypsin Deficiency.
Rueda JD, Sieluk J, Sandhaus RA, Mullins CD.
Value Health Reg Issues. 2020 Dec;23:70-76. doi: 10.1016/j.vhri.2020.04.003. Epub 2020 Sep 3.
PMID: 32892111

Biomarker-guided preemption of steroid-refractory graft-versus-host disease with α-1-antitrypsin.
Gergoudis SC, DeFilipp Z, Özbek U, Sandhu KS, Etra AM, Choe HK, Kitko CL, Ayuk F, Aziz M, Baez J, Ben-David K, Bunworasate U, Gandhi I, Hexner EO, Hogan WJ, Holler E, Kasikis S, Kowalyk SM, Lin JY, Merli P, Morales G, Nakamura R, Reshef R, Rösler W, Srinagesh H, Young R, Chen YB, Ferrara JLM, Levine JE.
Blood Adv. 2020 Dec 22;4(24):6098-6105. doi: 10.1182/bloodadvances.2020003336.
PMID: 33351103 Free PMC article.

Alpha-1 Antitrypsin Augmentation Therapy Improves Survival in Severely Deficient Patients with Predicted FEV1 Between 10% and 60%: A Retrospective Analysis of the NHLBI Alpha-1 Antitrypsin Deficiency Registry.
Rahaghi FF, Monk R, Ramakrishnan V, Beiko T, Strange C. Int J Chron Obstruct Pulmon Dis. 2020 Dec 3;15:3193-3199. doi: 10.2147/COPD.S263725. eCollection 2020.
PMID: 33299307 Free PMC article.

Serum Levels of Glutamate-Pyruvate Transaminase, Glutamate-Oxaloacetate Transaminase and Gamma-Glutamyl Transferase in 1494 Patients with Various Genotypes for the Alpha-1 Antitrypsin Gene.
Hernández Pérez JM, Blanco I, Jesús Sánchez Medina A, Díaz Hernández L, Antonio Pérez Pérez J.
J Clin Med. 2020 Dec 3;9(12):3923. doi: 10.3390/jcm9123923.
PMID: 33287251 Free PMC article.

Can Alpha-1 Antitrypsin Levels be Used to Predict the Prognosis of COVID-19 Therapy?
Aras Atik E, Özdemir N, Demirkan K. Turk J Pharm Sci. 2020 Dec 23;17(6):576-577. doi: 10.4274/tjps.galenos.2020.20727.
PMID: 33389945 Free PMC article. No abstract available.

The Pi*MZ Allele in Alpha-1 Antitrypsin Increases Liver-Related Outcomes in a Population-Based Study.
Luukkonen PK, Salomaa V, Åberg F.
Gastroenterology. 2020 Dec 29:S0016-5085(20)35596-7. doi: 10.1053/j.gastro.2020.10.061. Online ahead of print.
PMID: 33385428 No abstract available.

An auspicious triumph of recovery from dialysis-requiring acute kidney injury in COVID-19 in a patient with chronic kidney disease, α-1 antitrypsin deficiency, and liver transplant: A case report.
De Souza L, Nwanji V, Kaur G. Clin Nephrol. 2020 Dec;94(6):297-306. doi: 10.5414/CN110294.
PMID: 32909541

November 2020 Publications

Ethnic differences in alpha-1 antitrypsin deficiency allele frequencies may partially explain national differences in COVID-19 fatality rates.
Shapira G, Shomron N, Gurwitz D.
FASEB J. 2020 Nov;34(11):14160-14165. doi: 10.1096/fj.202002097. Epub 2020 Sep 22.
PMID: 32960480

Alpha-1 antitrypsin deficiency and risk of lung cancer: A systematic review.
Tubío-Pérez RA, Torres-Durán M, Fernández-Villar A, Ruano-Raviña A.Transl Oncol. 2021 Jan;14(1):100914. doi: 10.1016/j.tranon.2020.100914. Epub 2020 Nov 1. PMID: 33142121

Advances in Alpha-1 Antitrypsin Gene Therapy. Lorincz R, Curiel DT.
Am J Respir Cell Mol Biol. 2020 Nov;63(5):560-570. doi: 10.1165/rcmb.2020-0159PS. PMID: 32668173 Review.

Alpha-1 antitrypsin for cystic fibrosis complicated by severe cytokinemic COVID-19.
McElvaney OJ, O’Connor E, McEvoy NL, Fraughan DD, Clarke J, McElvaney OF, Gunaratnam C, O’Rourke J, Curley GF, McElvaney NG.
J Cyst Fibros. 2020 Nov 20:S1569-1993(20)30915-2. doi: 10.1016/j.jcf.2020.11.012. Online ahead of print.
PMID: 33288475 Free PMC article.

Alpha-1 antitrypsin deficiency is under-recognized in individuals with cirrhosis undergoing liver transplantation.
Shah RS, Alsuleiman B, Bena J, Stoller JK, Wakim-Fleming J. Eur J Gastroenterol Hepatol. 2020 Nov 30. doi: 10.1097/MEG.0000000000002005. Online ahead of print. PMID: 33252420

Alpha 1 Antitrypsin-Deficient Macrophages Have Impaired Efferocytosis of Apoptotic Neutrophils.
Lee J, Lu Y, Oshins R, West J, Moneypenny CG, Han K, Brantly ML. Front Immunol. 2020 Nov 20;11:574410. doi: 10.3389/fimmu.2020.574410. eCollection 2020. PMID: 33329539 Free PMC article.

The Distribution of Alpha-1 Antitrypsin Genotypes Between Patients with COPD/Emphysema, Asthma and Bronchiectasis.
Veith M, Tüffers J, Peychev E, Klemmer A, Kotke V, Janciauskiene S, Wilhelm S, Bals R, Koczulla AR, Vogelmeier CF, Greulich T. Int J Chron Obstruct Pulmon Dis. 2020 Nov 6;15:2827-2836. doi: 10.2147/COPD.S271810. eCollection 2020. PMID: 33192056 Free PMC article.

Exacerbations of Lung Disease in Alpha-1 Antitrypsin Deficiency.
Smith DJ, Ellis PR, Turner AM. Chronic Obstr Pulm Dis. 2020 Nov 25. doi: 10.15326/jcopdf.2020.0173. Online ahead of print. PMID: 33238089

Hypothesis: Alpha-1-antitrypsin is a promising treatment option for COVID-19.
Bai X, Hippensteel J, Leavitt A, Maloney JP, Beckham D, Garcia C, Li Q, Freed BM, Ordway D, Sandhaus RA, Chan ED. Med Hypotheses. 2021 Jan;146:110394. doi: 10.1016/j.mehy.2020.110394. Epub 2020 Nov 12. PMID: 33239231 Free PMC article.

Recurrence of emphysema post-lung transplantation in a patient with alpha 1 antitrypsin deficiency (AATD).
Ataya A. Respir Med Case Rep. 2020 Nov 26;31:101309. doi: 10.1016/j.rmcr.2020.101309. eCollection 2020. PMID: 33304809

Renoprotective Effects of Alpha-1 Antitrypsin against Tacrolimus-Induced Renal Injury.
Lim JH, Oh EJ, Oh SH, Jung HY, Choi JY, Cho JH, Park SH, Kim YL, Kim CD. Int J Mol Sci. 2020 Nov 16;21(22):8628. doi: 10.3390/ijms21228628. PMID: 33207690

Human plasma-derived alpha1 -proteinase inhibitor in patients with new-onset type 1 diabetes mellitus: A randomized, placebo-controlled proof-of-concept study.
Lagarde WH, Courtney KL, Reiner B, Steinmann K, Tsalikian E, Willi SM. Pediatr Diabetes. 2020 Nov 26. doi: 10.1111/pedi.13162. Online ahead of print. PMID: 33244872

Plasma Cytokeratin 18 and fecal Alpha-1 Antitrypsin concentrations in dogs with osteosarcoma receiving carboplatin chemotherapy.
Taikowski K, Rudinsky AJ, Louke DS, Warry E, Fenger JM. Vet Med Sci. 2020 Nov 22. doi: 10.1002/vms3.392. Online ahead of print. PMID: 33222415

[Research progress of hepatocyte transplantation treatment for alpha-1 antitrypsin deficiency].
Xu WJ, Xiao JF, Chen YM, Ding JQ. Zhonghua Gan Zang Bing Za Zhi. 2020 Nov 20;28(11):973-976. doi: 10.3760/cma.j.cn501113- 20201103-00593. PMID: 33256287 Chinese.

The Clinical Utility of Determining the Allelic Background of Mutations Causing Alpha-1 Antitrypsin Deficiency: The Case with the Null Variant Q0(Mattawa)/Q0(ourém).
Bellemare J, Gaudreault N, Valette K, Belmonte I, Nuñez A, Miravitlles M, Maltais F, Bossé Y. Chronic Obstr Pulm Dis. 2020 Nov 2. doi: 10.15326/jcopdf.8.1.2020.0168. Online ahead of print. PMID: 33150777

Disseminated panniculitis with subcutaneous abscesses and fat necrosis associated with alpha-1 antitrypsin deficiency.
Schöfl S, Koch-Thalhammer D, Jabkowski J, Sega W, Sepp N, Geroldinger-Simic M.J Dtsch Dermatol Ges. 2020 Nov;18(11):1309-1311. doi: 10.1111/ddg.14152. Epub 2020 Jul 12. PMID: 32657012 No abstract available.

A rare case of pulmonary disease combining alpha-1-antitrypsin deficiency and common variable immunodeficiency.
Sousa CS, Teixeira V, Pereira V, Pinheiro RB, Seixas S, Martins N. Pulmonology. 2020 Nov-Dec;26(6):406-409. doi: 10.1016/j.pulmoe.2020.04.016. Epub 2020 Jun 6. PMID: 32518030 No abstract available.

Panniculitis as main clinical manifestation of alpha-1 antitrypsin deficiency revealing a SerpinA1 gene mutation.
Abdalla BMZ, Criado PR. Australas J Dermatol. 2020 Nov;61(4):e470-e471. doi: 10.1111/ajd.13332. Epub 2020 May 19. PMID: 32430912 No abstract available.

Prevalence of Anti-Neutrophil Cytoplasmic Antibodies and Associated Vasculitis in COPD Associated With Alpha-1 Antitrypsin Deficiency: An Ancillary Study to a Prospective Study on 180 French Patients.
Deshayes S, Martin Silva N, Khoy K, Mariotte D, Le Mauff B, Mornex JF, Pison C, Cuvelier A, Balduyck M, Pujazon MC, Fournier M, Ait Ilalne B, Thabut G, Mal H, Aouba A. Chest. 2020 Nov;158(5):1919-1922. doi: 10.1016/j.chest.2020.04.054. Epub 2020 May 14. PMID: 32417149 No abstract available.

October 2020 Publications

The structural basis for Z α1-antitrypsin polymerization in the liver.
Faull SV, Elliston ELK, Gooptu B, Jagger AM, Aldobiyan I, Redzej A, Badaoui M, Heyer-Chauhan N, Rashid ST, Reynolds GM, Adams DH, Miranda E, Orlova EV, Irving JA, Lomas DA.
Sci Adv. 2020 Oct 21;6(43):eabc1370. doi: 10.1126/sciadv.abc1370. Print 2020 Oct.
PMID: 33087346 Free PMC article.

Alpha-1 antitrypsin deficiency and risk of lung cancer: A systematic review.
Tubío-Pérez RA, Torres-Durán M, Fernández-Villar A, Ruano-Raviña A.
Transl Oncol. 2020 Oct 31;14(1):100914. doi: 10.1016/j.tranon.2020.100914. Online ahead of print.
PMID: 33142121

Salivary alpha-1-antitrypsin and macrophage migration inhibitory factor may be potential prognostic biomarkers for oncologic treatment-induced severe oral mucositis.
Palmier NR, Leme AFP, De Rossi T, Telles GP, Morais-Faria K, Kowalski LP, Marta GN, Brandão TB, Arany PR, Migliorati CA, Santos-Silva AR, Prado-Ribeiro AC.
Support Care Cancer. 2020 Oct 2. doi: 10.1007/s00520-020-05805-2. Online ahead of print.
PMID: 33009579

Alpha 1 Antitrypsin is an Inhibitor of the SARS-CoV-2-Priming Protease TMPRSS2.
Azouz NP, Klingler AM, Callahan V, Akhrymuk IV, Elez K, Raich L, Henry BM, Benoit JL, Benoit SW, Noé F, Kehn-Hall K, Rothenberg ME.
bioRxiv. 2020 Oct 7:2020.05.04.077826. doi: 10.1101/2020.05.04.077826. Preprint.
PMID: 33052338 Free PMC article.

Trends in Diagnosis of Alpha-1 Antitrypsin Deficiency Between 2015 and 2019 in a Reference Laboratory.
Belmonte I, Nuñez A, Barrecheguren M, Esquinas C, Pons M, López-Martínez RM, Ruiz G, Blanco-Grau A, Ferrer R, Genescà J, Miravitlles M, Rodríguez-Frías F.
Int J Chron Obstruct Pulmon Dis. 2020 Oct 7;15:2421-2431. doi: 10.2147/COPD.S269641. eCollection 2020.
PMID: 33116457 Free PMC article.

The molecular species responsible for α1 -antitrypsin deficiency are suppressed by a small molecule chaperone.
Ronzoni R, Heyer-Chauhan N, Fra A, Pearce AC, Rüdiger M, Miranda E, Irving JA, Lomas DA.
FEBS J. 2020 Oct 15. doi: 10.1111/febs.15597. Online ahead of print.
PMID: 33058391

Clinical outcomes and survival following lung transplantation in patients with Alpha-1 antitrypsin deficiency.
Riley L, Lascano J.
Respir Med. 2020 Oct;172:106145. doi: 10.1016/j.rmed.2020.106145. Epub 2020 Sep 2.
PMID: 32911139

Alpha-1 antitrypsin deficiency is significantly associated with atopy in asthmatic patients.
Aiello M, Frizzelli A, Pisi R, Fantin A, Ghirardini M, Marchi L, Ferrarotti I, Bertorelli G, Percesepe A, Chetta A.
J Asthma. 2020 Oct 19:1-8. doi: 10.1080/02770903.2020.1827421. Online ahead of print.
PMID: 32962473

Comorbidity Associations with AATD Among Commercially Insured and Medicare Beneficiaries with COPD in the US.
Sandhaus R, Strange C, Stone G, Runken MC, Blanchette CM, Howden R.
Int J Chron Obstruct Pulmon Dis. 2020 Oct 5;15:2389-2397. doi: 10.2147/COPD.S263297. eCollection 2020.
PMID: 33116454 Free PMC article.

Alpha-1 Antitrypsin Suppresses Macrophage Activation and Promotes Islet Graft Survival after Intrahepatic Islet Transplantation.
Gou W, Wang J, Song L, Kim DS, Cui W, Strange C, Wang H.
Am J Transplant. 2020 Oct 12. doi: 10.1111/ajt.16342. Online ahead of print.
PMID: 33047509

Bench to Bedside and Back: The Evolving Story of Alpha-1 Antitrypsin Deficiency.
Chapman KR.
Am J Respir Cell Mol Biol. 2020 Oct;63(4):403-404. doi: 10.1165/rcmb.2020-0243ED.
PMID: 32716630 Free PMC article. No abstract available.

Modernising case finding for α1-antitrypsin deficiency by DNA sequencing of COPD patients.
Quinn M, Turner AM.
Eur Respir J. 2020 Oct 15;56(4):2002628. doi: 10.1183/13993003.02628-2020. Print 2020 Oct.
PMID: 33060164 No abstract available.

New cis-Acting Variants in PI*S Background Produce Null Phenotypes Causing Alpha-1 Antitrypsin Deficiency.
Matamala N, Gomez-Mariano G, Perez JA, Baladrón B, Torres-Durán M, Michel FJ, Saez R, Hernández-Pérez JM, Belmonte I, Rodriguez-Frias F, Blanco I, Strnad P, Janciauskiene S, Martinez-Delgado B.
Am J Respir Cell Mol Biol. 2020 Oct;63(4):444-451. doi: 10.1165/rcmb.2020-0021OC.
PMID: 32515985

Home-Based Multicomponent Intervention Increases Exercise Activity and Improves Body Mass Index: Results of a 5-Year Randomized Trial Among Individuals with Alpha-1 Antitrypsin Deficiency-Associated Lung Disease.
Choate R, Mannino DM, Holm KE, Beiko T, Boyd B, Sandhaus RA.
Chronic Obstr Pulm Dis. 2020 Oct 30. doi: 10.15326/jcopdf.8.1.2020.0183. Online ahead of print.
PMID: 33135406

Long term results of liver transplantation for alpha-1 antitrypsin deficiency.
Guillaud O, Jacquemin E, Couchonnal E, Vanlemmens C, Francoz C, Chouik Y, Conti F, Duvoux C, Hilleret MN, Kamar N, Houssel-Debry P, Neau-Cransac M, Pageaux GP, Gonzales E, Ackermann O, Gugenheim J, Lachaux A, Ruiz M, Radenne S, Debray D, Lacaille F, McLin V, Duclos-Vallée JC, Samuel D, Coilly A, Dumortier J.
Dig Liver Dis. 2020 Oct 30:S1590-8658(20)30970-1. doi: 10.1016/j.dld.2020.10.016. Online ahead of print.
PMID: 33139195

Alpha 1-Antitrypsin deficiency in liver explants in a Mexican cohort: A unique cohort to assess the role of heterozygous genotypes in liver disease.
Karatas E, Bouchecareilh M.
Clin Res Hepatol Gastroenterol. 2020 Oct 14:S2210-7401(20)30276-X. doi: 10.1016/j.clinre.2020.09.003. Online ahead of print.
PMID: 33069636 No abstract available.

September 2020 Publications

Prevalence of Alpha-1 Antitrypsin Deficiency in COPD Patients in Argentina. The DAAT.AR Study.
Menga G, Fernandez Acquier M, Echazarreta AL, Sorroche PB, Lorenzon MV, Fernández ME, Saez MS; grupo de estudio DAAT.AR.
Arch Bronconeumol. 2020 Sep;56(9):571-577. doi: 10.1016/j.arbres.2019.11.010. Epub 2019 Dec 27.
PMID: 31889566 English, Spanish.

Geographical Overlap Between Alpha-1 Antitrypsin Deficiency and COVID-19 Infection in Italy: Casual or Causal?
Vianello A, Braccioni F.
Arch Bronconeumol. 2020 Sep;56(9):609-610. doi: 10.1016/j.arbres.2020.05.015. Epub 2020 May 31.
PMID: 32576394 English, Spanish. No abstract available.

SZ alpha-1 antitrypsin deficiency and pulmonary disease: more like MZ, not like ZZ.
Franciosi AN, Carroll TP, McElvaney NG.
Thorax. 2020 Sep 11:thoraxjnl-2020-215250. doi: 10.1136/thoraxjnl-2020-215250. Online ahead of print.
PMID: 32917839

Exploiting the Disialyl Galactose Activity of α2,6-Sialyltransferase from Photobacterium damselae To Generate a Highly Sialylated Recombinant α-1-Antitrypsin.
Pallister EG, Choo MSF, Tai JN, Leong DSZ, Tang WQ, Ng SK, Huang K, Marchesi A, Both P, Gray C, Rudd PM, Flitsch SL, Nguyen-Khuong T.
Biochemistry. 2020 Sep 1;59(34):3123-3128. doi: 10.1021/acs.biochem.9b00563. Epub 2019 Oct 11.
PMID: 31580652

Homocysteinylated alpha 1 antitrypsin as an antigenic target of autoantibodies in seronegative rheumatoid arthritis patients.
Colasanti T, Sabatinelli D, Mancone C, Giorgi A, Pecani A, Spinelli FR, Di Giamberardino A, Navarini L, Speziali M, Vomero M, Barbati C, Perricone C, Ceccarelli F, Finucci A, Celia AI, Currado D, Afeltra A, Schininà ME, Barnaba V, Conti F, Valesini G, Alessandri C.
J Autoimmun. 2020 Sep;113:102470. doi: 10.1016/j.jaut.2020.102470. Epub 2020 May 28.
PMID: 32473759

Alpha-1 Antitrypsin Deficiency Associated COPD.
Strange C.
Clin Chest Med. 2020 Sep;41(3):339-345. doi: 10.1016/j.ccm.2020.05.003.
PMID: 32800189 Review.

Limitations and Challenges of Conducting Budget Impact Analyses in Rare Diseases: A Case Study of Alpha-1 Antitrypsin Deficiency.
Rueda JD, Sieluk J, Sandhaus RA, Mullins CD.
Value Health Reg Issues. 2020 Sep 3;23:70-76. doi: 10.1016/j.vhri.2020.04.003. Online ahead of print.
PMID: 32892111

Prevalence of α1-antitrypsin PiZZ genotypes in patients with COPD in Europe: a systematic review.
Blanco I, Diego I, Bueno P, Pérez-Holanda S, Casas-Maldonado F, Miravitlles M.
Eur Respir Rev. 2020 Jul 21;29(157):200014. doi: 10.1183/16000617.0014-2020. Print 2020 Sep 30.
PMID: 32699024 Review.

Evaluation of the Small Airways in Patients with Chronic Obstructive Pulmonary Disease and Alpha-1 Antitrypsin Deficiency.
Ferrari da Cruz T, Rufino R, Lopes AJ, Noronha A, Medeiros Anselmo F, Henrique da Costa C.
Int J Chron Obstruct Pulmon Dis. 2020 Sep 24;15:2267-2274. doi: 10.2147/COPD.S262418. eCollection 2020.
PMID: 33061344 Free PMC article.

CHOP and c-JUN up-regulate the mutant Z α1-antitrypsin, exacerbating its aggregation and liver proteotoxicity.
Attanasio S, Ferriero R, Gernoux G, De Cegli R, Carissimo A, Nusco E, Campione S, Teckman J, Mueller C, Piccolo P, Brunetti-Pierri N.
J Biol Chem. 2020 Sep 18;295(38):13213-13223. doi: 10.1074/jbc.RA120.014307. Epub 2020 Jul 28.
PMID: 32723872

Overexpression of alpha-1 antitrypsin in mesenchymal stromal cells improves their intrinsic biological properties and therapeutic effects in nonobese diabetic mice.
Song L, Gou W, Wang J, Wei H, Lee J, Strange C, Wang H.
Stem Cells Transl Med. 2020 Sep 18. doi: 10.1002/sctm.20-0122. Online ahead of print.
PMID: 32945622

Calreticulin enhances the secretory trafficking of a misfolded alpha-1-antitrypsin.
Mohan HM, Yang B, Dean NA, Raghavan M.
J Biol Chem. 2020 Sep 25:jbc.RA120.014372. doi: 10.1074/jbc.RA120.014372. Online ahead of print.
PMID: 32978262

Medical costs of Alpha-1 antitrypsin deficiency-associated COPD in the United States.
Sieluk J, Slejko JF, Silverman H, Perfetto E, Mullins CD.
Orphanet J Rare Dis. 2020 Sep 23;15(1):260. doi: 10.1186/s13023-020-01523-4.
PMID: 32967697 Free PMC article.

Alpha-1 antitrypsin deficient individuals have circulating extracellular vesicles with profibrogenic cargo.
Khodayari N, Oshins R, Holliday LS, Clark V, Xiao Q, Marek G, Mehrad B, Brantly M.
Cell Commun Signal. 2020 Sep 4;18(1):140. doi: 10.1186/s12964-020-00648-0.
PMID: 32887613 Free PMC article.

An auspicious triumph of recovery from dialysis-requiring acute kidney injury in COVID-19 in a patient with chronic kidney disease, α-1 antitrypsin deficiency, and liver transplant: A case report.
De Souza L, Nwanji V, Kaur G.
Clin Nephrol. 2020 Sep 10. doi: 10.5414/CN110294. Online ahead of print.
PMID: 32909541

Correction to: Alpha-1 Antitrypsin Deficiency: a Rare Disease?
Cortes-Lopez R, Barjaktarevic I.
Curr Allergy Asthma Rep. 2020 Sep 12;20(11):71. doi: 10.1007/s11882-020-00966-w.
PMID: 32918642

Severe Nocardia pneumonia in an immunocompromised patient with alpha-1 antitrypsin deficiency.
Chan DECY, Hamed D, Lennon D, Wark P.
Respirol Case Rep. 2020 Sep 24;8(8):e00670. doi: 10.1002/rcr2.670. eCollection 2020 Nov.
PMID: 33005425 Free PMC article.

Correction: Phage Display of the Serpin Alpha-1 Proteinase Inhibitor Randomized at Consecutive Residues in the Reactive Centre Loop and Biopanned with or without Thrombin.
Scott BM, Matochko WL, Gierczak RF, Bhakta V, Derda R, Sheffield WP.
PLoS One. 2020 Sep 10;15(9):e0238969. doi: 10.1371/journal.pone.0238969. eCollection 2020.
PMID: 32913368 Free PMC article.

August 2020 Publications

Alpha 1 Antitrypsin Deficiency.
Meseeha M, Attia M.
2020 Aug 10. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan–.
PMID: 28723059 Free Books & Documents. Review.

Alpha-1-antitrypsin: A possible host protective factor against Covid-19.
de Loyola MB, Dos Reis TTA, de Oliveira GXLM, da Fonseca Palmeira J, Argañaraz GA, Argañaraz ER.
Rev Med Virol. 2020 Aug 26:e2157. doi: 10.1002/rmv.2157. Online ahead of print.
PMID: 32844538 Free PMC article. Review.

In plain sight: the role of alpha-1-antitrypsin in COVID-19 pathogenesis and therapeutics.
Oguntuyo KY, Stevens CS, Siddiquey MN, Schilke RM, Woolard MD, Zhang H, Acklin JA, Ikegame S, Hung CT, Lim JK, Cross RW, Geisbert TW, Ivanov SS, Kamil JP, Lee B.
bioRxiv. 2020 Aug 15:2020.08.14.248880. doi: 10.1101/2020.08.14.248880. Preprint.
PMID: 32817940 Free PMC article.

Gene therapy for alpha 1-antitrypsin deficiency with an oxidant-resistant human alpha 1-antitrypsin.
Sosulski ML, Stiles KM, Frenk EZ, Hart FM, Matsumura Y, De BP, Kaminsky SM, Crystal RG.
JCI Insight. 2020 Aug 6;5(15):e135951. doi: 10.1172/jci.insight.135951.
PMID: 32759494 Free PMC article.

The Importance of Reference Centers and Registries for Rare Diseases: The Example of Alpha-1 Antitrypsin Deficiency.
Miravitlles M, Nuñez A, Torres-Durán M, Casas-Maldonado F, Rodríguez-Hermosa JL, López-Campos JL, Calle M, Rodríguez E, Esquinas C, Barrecheguren M.
COPD. 2020 Aug;17(4):346-354. doi: 10.1080/15412555.2020.1795824.
PMID: 32791925

PredictAAT: Accounting for Inflammation in the Diagnosis of Alpha 1 Antitrypsin Deficiency.
Kueppers F.
COPD. 2020 Aug 30:1-4. doi: 10.1080/15412555.2020.1812055. Online ahead of print.
PMID: 32862719

Decrease in alpha-1 antiproteinase antitrypsin is observed in primary Sjogren’s syndrome condition.
Singh BB, Ohm J, Quenum Zanbede FO, Chauhan P, Kroese FGM, Vissink A, Ambrus JL, Mishra BB.
Autoimmunity. 2020 Aug;53(5):270-282. doi: 10.1080/08916934.2020.1768376. Epub 2020 May 25.
PMID: 32449389

Is Heterozygosity for the Alpha-1 Antitrypsin Risk Allele Pi∗MZ a Disease Modifier or Genetic Risk Factor?
Zoller H, Wagner S, Tilg H.
Gastroenterology. 2020 Aug;159(2):433-434. doi: 10.1053/j.gastro.2020.06.035. Epub 2020 Jun 20.
PMID: 32569768 No abstract available.

Identification of creatine kinase and alpha-1 antitrypsin as protein targets of alkylation by sulfur mustard.
Lüling R, Schmeißer W, Siegert M, Mückter H, Dietrich A, Thiermann H, Gudermann T, John H, Steinritz D.
Drug Test Anal. 2020 Aug 27. doi: 10.1002/dta.2916. Online ahead of print.
PMID: 32852113

July 2020 Publications

Alpha-1 Antitrypsin Deficiency: Have We Got the Right Proteinase?
Stockley RA.
Chronic Obstr Pulm Dis. 2020 Jul;7(3):163-171. doi: 10.15326/jcopdf.7.3.2019.0151.
PMID: 32396717 Review.

Accelerated 129 Xe MRI morphometry of terminal airspace enlargement: Feasibility in volunteers and those with alpha-1 antitrypsin deficiency.
Ouriadov A, Guo F, McCormack DG, Parraga G.
Magn Reson Med. 2020 Jul;84(1):416-426. doi: 10.1002/mrm.28091. Epub 2019 Nov 25.
PMID: 31765497

Intrahepatic heteropolymerization of M and Z alpha-1-antitrypsin.
Laffranchi M, Elliston EL, Miranda E, Perez J, Ronzoni R, Jagger AM, Heyer-Chauhan N, Brantly ML, Fra A, Lomas DA, Irving JA.
JCI Insight. 2020 Jul 23;5(14):e135459. doi: 10.1172/jci.insight.135459.
PMID: 32699193 Free PMC article.

Neutrophil Modulation in Alpha-1 Antitrypsin Deficiency.
Sapey E.
Chronic Obstr Pulm Dis. 2020 Jul;7(3):247-259. doi: 10.15326/jcopdf.7.3.2019.0164.
PMID: 32697897 Review.

Alpha-1 Antitrypsin Deficiency: The Learning Goes On.
Stockley RA.
Am J Respir Crit Care Med. 2020 Jul 1;202(1):6-7. doi: 10.1164/rccm.202004-0922ED.
PMID: 32343597 Free PMC article. No abstract available.

Clarifying the Risk of Lung Disease in SZ Alpha-1 Antitrypsin Deficiency.
Franciosi AN, Hobbs BD, McElvaney OJ, Molloy K, Hersh C, Clarke L, Gunaratnam C, Silverman EK, Carroll TP, McElvaney NG.
Am J Respir Crit Care Med. 2020 Jul 1;202(1):73-82. doi: 10.1164/rccm.202002-0262OC.
PMID: 32197047

Utility of routine screening for alpha-1 antitrypsin deficiency in patients with bronchiectasis.
Carreto L, Morrison M, Donovan J, Finch S, Tan GL, Fardon T, Wilson R, Furrie E, Loebinger M, Chalmers JD.
Thorax. 2020 Jul;75(7):592-593. doi: 10.1136/thoraxjnl-2019-214195. Epub 2020 Apr 17.
PMID: 32303623 Free PMC article. Review.

The Undiagnosed Disease Burden Associated with Alpha-1 Antitrypsin Deficiency Genotypes.
Nakanishi T, Forgetta V, Handa T, Hirai T, Mooser V, Lathrop MG, Cookson WOCM, Richards JB.
Eur Respir J. 2020 Jul 16:2001441. doi: 10.1183/13993003.01441-2020. Online ahead of print.
PMID: 32675199

[Alpha-1 Antitrypsin Deficiency (AATD) – D-A-CH-Expert Statement].
Greulich T, Fähndrich S, Clarenbach C, Gleiber W, Hautmann H, Heine R, Idzko M, Schmidt-Scherzer K, Skowasch D, Wiewrodt R, Bals R, Koczulla AR.
Pneumologie. 2020 Jul;74(7):436-442. doi: 10.1055/a-1143-8186. Epub 2020 Jun 3.
PMID: 32492720 German. No abstract available.

Anti-cytokines as a Strategy in Alpha-1 Antitrypsin Deficiency.
McElvaney OF, Murphy MP, Reeves EP, McElvaney NG.
Chronic Obstr Pulm Dis. 2020 Jul;7(3):203-213. doi: 10.15326/jcopdf.7.3.2019.0171.
PMID: 32503090 Review.

Alpha 1 Antitrypsin Mutation.
Abdulkarim A, Craig TJ.
2020 Jul 15. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan–.
PMID: 29489299 Free Books & Documents. Review.

Leveraging Population Genomics for Individualized Correction of the Hallmarks of Alpha-1 Antitrypsin Deficiency.
Wang C, Zhao P, Sun S, Teckman J, Balch WE.
Chronic Obstr Pulm Dis. 2020 Jul;7(3):224-246. doi: 10.15326/jcopdf.7.3.2019.0167.
PMID: 32726074 Review.

Alpha-1 Antitrypsin Deficiency-Mediated Liver Toxicity: Why Do Some Patients Do Poorly? What Do We Know So Far?
Bouchecareilh M.
Chronic Obstr Pulm Dis. 2020 Jul;7(3):172-181. doi: 10.15326/jcopdf.7.3.2019.0148.
PMID: 32558486 Review.

Longitudinal Outcomes in Young Patients with Alpha-1-Antitrypsin Deficiency with Native Liver Reveal that Neonatal Cholestasis is a Poor Predictor of Future Portal Hypertension.
Teckman J, Rosenthal P, Hawthorne K, Spino C, Bass LM, Murray KF, Kerkar N, Magee JC, Karpen S, Heubi JE, Molleston JP, Squires RH, Kamath BM, Guthery SL, Loomes KM, Sherker AH, Sokol RJ; ChiLDReN (Childhood Liver Disease Research Network).
J Pediatr. 2020 Jul 11:S0022-3476(20)30874-X. doi: 10.1016/j.jpeds.2020.07.031. Online ahead of print.
PMID: 32663593

Association of α 1 Antitrypsin Phenotype and Development of Advanced Liver Disease and Pulmonary Complications Before and After Liver Transplantation.
Abu Rmilah A, Fencl R, Watt K, Krowka M, Wiesner R, Murray D, Nyberg S, Leise M.
Transplantation. 2020 Jul 6. doi: 10.1097/TP.0000000000003390. Online ahead of print.
PMID: 32732616

Feasibility of Aerosolized Alpha-1 Antitrypsin as a Therapeutic Option.
Usmani OS.
Chronic Obstr Pulm Dis. 2020 Jul;7(3):272-279. doi: 10.15326/jcopdf.7.3.2019.0179.
PMID: 32726075 Review.

A Highly Phenotyped Open Access Repository of Alpha-1 Antitrypsin Deficiency Pluripotent Stem Cells.
Kaserman JE, Hurley K, Dodge M, Villacorta-Martin C, Vedaie M, Jean JC, Liberti DC, James MF, Higgins MI, Lee NJ, Washko GR, San Jose Estepar R, Teckman J, Kotton DN, Wilson AA.
Stem Cell Reports. 2020 Jul 14;15(1):242-255. doi: 10.1016/j.stemcr.2020.06.006. Epub 2020 Jul 2.
PMID: 32619491 Free PMC article.

The Alpha-1 Antitrypsin Polymer Load Correlates With Hepatocyte Senescence, Fibrosis Stage and Liver-Related Mortality.
Mela M, Smeeton W, Davies SE, Miranda E, Scarpini C, Coleman N, Alexander GJM.
Chronic Obstr Pulm Dis. 2020 Jul;7(3):151-162. doi: 10.15326/jcopdf.7.3.2019.0158.
PMID: 32726073

Alpha-1 antitrypsin governs alcohol-related liver disease in mice and humans.
Grander C, Schaefer B, Schwärzler J, Grabherr F, de Graaf DM, Enrich B, Oberhuber G, Mayr L, Sangineto M, Jaschke N, Adolph TE, Effenberger M, Moschen AR, Dinarello CA, Zoller H, Tilg H.
Gut. 2020 Jul 22:gutjnl-2020-321375. doi: 10.1136/gutjnl-2020-321375. Online ahead of print.
PMID: 32699098

Why is Disease Penetration so Variable in Alpha-1 Antitrypsin Deficiency? The Contribution of Environmental Factors.
Rangaraju M, Turner AM.
Chronic Obstr Pulm Dis. 2020 Jul;7(3):280-289. doi: 10.15326/jcopdf.7.3.2019.0177.
PMID: 32698254 Review.

Why is Disease Penetration So Variable? Role of Genetic Modifiers of Lung Function in Alpha-1 Antitrypsin Deficiency.
Hobbs BD, Cho MH.
Chronic Obstr Pulm Dis. 2020 Jul;7(3):214-223. doi: 10.15326/jcopdf.7.3.2019.0159.
PMID: 32621460 Review.

Alpha-1-antitrypsin deficiency (carrier) as possible risk factor for development of colonic diverticula. A multicentre prospective case-control study: the ALADDIN study.
Rottier SJ, Dreuning LC, van Pelt J, van Geloven AAW, Beele XDY, Huisman PM, Deurholt WY, Rottier CA, van Leeuwen K, de Boer M, van Mierlo G, Boermeester MA, Schreurs WH; ALADDIN Collaborative Study Group.
Colorectal Dis. 2020 Jul 15. doi: 10.1111/codi.15270. Online ahead of print.
PMID: 32666625

Towards New Therapeutic Solutions for Alpha-1 Antitrypsin Deficiency: Role of the Alpha-1 Foundation.
Wanner A.
Chronic Obstr Pulm Dis. 2020 Jul;7(3):147-150. doi: 10.15326/jcopdf.7.3.2020.0141.
PMID: 32726072 No abstract available.

Alpha-1-antitrypsin molecular testing in Canada: A seven year, multi-centre comparison.
Mattman A, Gilfix BM, Chen SX, DeMarco ML, Kyle BD, Parker ML, Agbor TA, Jung B, Selvarajah S, Barakauskas VE, Vaags AK, Estey MP, Nelson TN, Speevak MD.
Clin Biochem. 2020 Jul;81:27-33. doi: 10.1016/j.clinbiochem.2020.05.001. Epub 2020 May 6.
PMID: 32387440

Non-Invasive Assessment and Management of Liver Involvement in Adults With Alpha-1 Antitrypsin Deficiency.
Hamesch K, Strnad P.
Chronic Obstr Pulm Dis. 2020 Jul;7(3):260-271. doi: 10.15326/jcopdf.7.3.2019.0161.
PMID: 32697896 Review.

Disseminated panniculitis with subcutaneous abscesses and fat necrosis associated with alpha-1 antitrypsin deficiency.
Schöfl S, Koch-Thalhammer D, Jabkowski J, Sega W, Sepp N, Geroldinger-Simic M.
J Dtsch Dermatol Ges. 2020 Jul 12. doi: 10.1111/ddg.14152. Online ahead of print.
PMID: 32657012 No abstract available.

A PI*MS is not always a PI*MS. An example of when genotyping for alpha-1 antitrypsin deficiency is necessary.
Martín T, Miravitlles M, Furtado ST.
Pulmonology. 2020 Jul 25:S2531-0437(20)30152-5. doi: 10.1016/j.pulmoe.2020.06.013. Online ahead of print.
PMID: 32723617 No abstract available.

June 2020 Publications

Alpha-1 Antitrypsin Deficiency: Principles of Care.
Rodrigues JF, Mineiro A, Reis A, Ventura DG, Fernandez-Llimos F, Costa F, Gomes J, Silva JM, Lopes P, Cordeiro CR.
Acta Med Port. 2020 Jun 1;33(6):433-439. doi: 10.20344/amp.12950. Epub 2020 May 31.
PMID: 32504520

Proteomic and genomic biomarkers for Non-Small Cell Lung Cancer: Peroxiredoxin, Haptoglobin, and Alpha-1 antitrypsin.
Najafi Z, Mohamadnia A, Ahmadi R, Mahmoudi M, Bahrami N, Khosravi A, Jamaati H, Tabarsi P, Kazem Pour Dizaji M, Shirian S.
Cancer Med. 2020 Jun;9(11):3974-3982. doi: 10.1002/cam4.3019. Epub 2020 Mar 30.
PMID: 32232956 Free PMC article.

Alpha-1 Antitrypsin Deficiency and Pregnancy.
Gaeckle NT, Stephenson L, Reilkoff RA.
COPD. 2020 Jun;17(3):326-332. doi: 10.1080/15412555.2020.1754778. Epub 2020 Apr 20.
PMID: 32308050

Clinical considerations in individuals with α1-antitrypsin PI*SZ genotype.
McElvaney GN, Sandhaus RA, Miravitlles M, Turino GM, Seersholm N, Wencker M, Stockley RA.
Eur Respir J. 2020 Jun 18;55(6):1902410. doi: 10.1183/13993003.02410-2019. Print 2020 Jun.
PMID: 32165400 Free PMC article. Review.

Development of an RNAi therapeutic for alpha-1-antitrypsin liver disease.
Wooddell CI, Blomenkamp K, Peterson RM, Subbotin VM, Schwabe C, Hamilton J, Chu Q, Christianson DR, Hegge JO, Kolbe J, Hamilton HL, Branca-Afrazi MF, Given BD, Lewis DL, Gane E, Kanner SB, Teckman JH.
JCI Insight. 2020 Jun 18;5(12):135348. doi: 10.1172/jci.insight.135348.
PMID: 32379724

Alpha-1 Antitrypsin Deficiency: a Rare Disease?
Cortes-Lopez R, Barjaktarevic I.
Curr Allergy Asthma Rep. 2020 Jun 22;20(9):51. doi: 10.1007/s11882-020-00942-4.
PMID: 32572624 Review.

Relationship of CT densitometry to lung physiological parameters and health status in alpha-1 antitrypsin deficiency: initial report of a centralised database of the NIHR rare diseases translational research collaborative.
Crossley D, Stockley J, Bolton CE, Hopkinson NS, Mahadeva R, Steiner M, Wilkinson T, Hurst JR, Gooptu B, Stockley RA.
BMJ Open. 2020 Jun 30;10(6):e036045. doi: 10.1136/bmjopen-2019-036045.
PMID: 32606060 Free PMC article.

Alpha-1 Antitrypsin Deficiency-Mediated Liver Toxicity: Why Do Some Patients Do Poorly? What Do We Know So Far?
Bouchecareilh M.
Chronic Obstr Pulm Dis. 2020 Jun 17;7(3). doi: 10.15326/jcopdf.7.3.2019.0148. Online ahead of print.
PMID: 32558486 Review.

New cis-acting Variants in PI*S Background Produce Null Phenotypes Causing Alpha-1 Antitrypsin Deficiency.
Matamala N, Gomez-Mariano G, Perez JA, Baladrón B, Torres-Durán M, Michel FJ, Saez R, Hernández-Pérez JM, Belmonte I, Rodriguez-Frias F, Blanco I, Strnad P, Janciauskiene S, Martinez-Delgado B.
Am J Respir Cell Mol Biol. 2020 Jun 9. doi: 10.1165/rcmb.2020-0021OC. Online ahead of print.
PMID: 32515985

Decreased Risk of Ischemic Heart Disease in Individuals with Severe Alpha 1-Antitrypsin Deficiency (PiZZ) in Comparison with the General Population.
Tanash H, Ekström M, Basil N, Rönmark E, Lindberg A, Piitulainen E.
Int J Chron Obstruct Pulmon Dis. 2020 Jun 4;15:1245-1252. doi: 10.2147/COPD.S247377. eCollection 2020.
PMID: 32606637 Free PMC article.

Anti-cytokines as a Strategy in Alpha-1 Antitrypsin Deficiency.
McElvaney OF, Murphy MP, Reeves EP, McElvaney NG.
Chronic Obstr Pulm Dis. 2020 Jun 5;7(3). doi: 10.15326/jcopdf.7.3.2019.0171. Online ahead of print.
PMID: 32503090 Review.

Identification of small molecules by screening a mixture-based scaffold compound library for treatment of alpha-1 antitrypsin deficiency.
Zhang X, Santos R, Debevec G, Li D, Schutte R, Pham K, Liu C, Ostrov DA, Giulianotti M.
Biochem Biophys Res Commun. 2020 Jun 18;527(1):317-323. doi: 10.1016/j.bbrc.2020.04.037. Epub 2020 May 11.
PMID: 32446387

Alpha-1-antitrypsin deficiency (AATD) and spontaneous pneumothorax: Guidelines do not recommend screening for AATD in patients with pneumothorax – What did we find in 10 years of clinical evidence?
Dias E, Farinha I, Costa F.
Pulmonology. 2020 Jun 14:S2531-0437(20)30131-8. doi: 10.1016/j.pulmoe.2020.05.014. Online ahead of print.
PMID: 32553825 No abstract available.

α1-antitrypsin PI*SZ genotype: a SERPINA1 deficiency haplotype with uncertain clinical and therapeutic implications.
Blanco I, Diego I.
Eur Respir J. 2020 Jun 18;55(6):2000713. doi: 10.1183/13993003.00713-2020. Print 2020 Jun.
PMID: 32554776 No abstract available.

A rare case of pulmonary disease combining alpha-1-antitrypsin deficiency and common variable immunodeficiency.
Sousa CS, Teixeira V, Pereira V, Pinheiro RB, Seixas S, Martins N.
Pulmonology. 2020 Jun 6:S2531-0437(20)30097-0. doi: 10.1016/j.pulmoe.2020.04.016. Online ahead of print.
PMID: 32518030 No abstract available.

Update on Alpha-1 Antitrypsin Deficiency in Liver Disease.
Narayanan P, Mistry PK.
Clin Liver Dis (Hoboken). 2020 Jun 30;15(6):228-235. doi: 10.1002/cld.896. eCollection 2020 Jun.
PMID: 32617155 Free PMC article. Review. No abstract available.

Serum alpha-1 antitrypsin in acute ischemic stroke: A prospective pilot study.
Mahta A, Yaghi S, Reznik ME, Thompson BB, Wendell LC, Rao S, Potter NS, Dakay KB, Cutting S, Mac Grory B, Burton T, Saad A, Sacchetti DC, Stretz C, El Jamal S, Mahmoud LN, Moody S, Murray K, Costa S, Sellke FW, Kamel H, Furie KL.
J Clin Neurosci. 2020 Jun;76:20-24. doi: 10.1016/j.jocn.2020.04.074. Epub 2020 Apr 20.
PMID: 32327380

Post-Transcriptional Regulation of Alpha One Antitrypsin by a Proteasome Inhibitor.
Rao L, Xu Y, Reineke LC, Bhattacharya A, Tyryshkin A, Shin JN, Eissa NT.
Int J Mol Sci. 2020 Jun 17;21(12):4318. doi: 10.3390/ijms21124318.
PMID: 32560429 Free PMC article.

Quantitative Analysis of α-1-Antitrypsin Glycosylation Isoforms in HCC Patients Using LC-HCD-PRM-MS.
Yin H, Zhu J, Wang M, Yao ZP, Lubman DM.
Anal Chem. 2020 Jun 16;92(12):8201-8208. doi: 10.1021/acs.analchem.0c00420. Epub 2020 Jun 2.
PMID: 32426967

Is heterozygosity for the alpha-1 antitrypsin risk allele Pi*MZ disease modifier or genetic risk factor?
Zoller H, Wagner S, Tilg H.
Gastroenterology. 2020 Jun 19:S0016-5085(20)34835-6. doi: 10.1053/j.gastro.2020.06.035. Online ahead of print.
PMID: 32569768 No abstract available.

May 2020 Publications

Alpha-1 Antitrypsin Deficiency.
Stoller JK, Hupertz V, Aboussouan LS.
2006 Oct 27 [updated 2020 May 21]. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2020.
PMID: 20301692 Free Books & Documents. Review.

Alpha 1 Antitrypsin Deficiency.
Meseeha M, Attia M.
2020 May 18. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan–.
PMID: 28723059 Free Books & Documents. Review.

Alpha-1 Antitrypsin Deficiency: Have We Got the Right Proteinase?
Stockley RA.
Chronic Obstr Pulm Dis. 2020 May 12;7(3). doi: 10.15326/jcopdf.7.3.2019.0151. Online ahead of print.
PMID: 32396717 Review.

Decrease in alpha-1 antiproteinase antitrypsin is observed in primary Sjogren’s syndrome condition.
Singh BB, Ohm J, Quenum Zanbede FO, Chauhan P, Kroese FGM, Vissink A, Ambrus JL, Mishra BB.
Autoimmunity. 2020 May 25:1-13. doi: 10.1080/08916934.2020.1768376. Online ahead of print.
PMID: 32449389

Alpha-1 Antitrypsin Deficiency and Pulmonary Morbidity in Patients with Primary Immunodeficiency Disease: A Single-Center Experience.
Evers G, Schulze AB, Thrull M, Hering JP, Schülke C, Wiewrodt R, Wittkowski H, Schmidt LH, Mohr M.
Can Respir J. 2020 May 27;2020:4019608. doi: 10.1155/2020/4019608. eCollection 2020.
PMID: 32566054 Free PMC article.

α-1 Antitrypsin Genotype-Phenotype Discrepancy in a 42-Year-Old Man Who Carries the Null-Allele.
Pavičić T, Ćelap I, Njegovan M, Tešija Kuna A, Štefanović M.
Lab Med. 2020 May 6;51(3):301-305. doi: 10.1093/labmed/lmz059.
PMID: 31583408

Alpha-1 Antitrypsin Induces Epithelial-to-Mesenchymal Transition, Endothelial-to-Mesenchymal Transition, and Drug Resistance in Lung Cancer Cells.
Wu DM, Liu T, Deng SH, Han R, Zhang T, Li J, Xu Y.
Onco Targets Ther. 2020 May 4;13:3751-3763. doi: 10.2147/OTT.S242579. eCollection 2020.
PMID: 32440144 Free PMC article.

Homocysteinylated alpha 1 antitrypsin as an antigenic target of autoantibodies in seronegative rheumatoid arthritis patients.
Colasanti T, Sabatinelli D, Mancone C, Giorgi A, Pecani A, Spinelli FR, Di Giamberardino A, Navarini L, Speziali M, Vomero M, Barbati C, Perricone C, Ceccarelli F, Finucci A, Celia AI, Currado D, Afeltra A, Schininà ME, Barnaba V, Conti F, Valesini G, Alessandri C.
J Autoimmun. 2020 May 27:102470. doi: 10.1016/j.jaut.2020.102470. Online ahead of print.
PMID: 32473759

NullCanada: A novel α1-antitrypsin allele with in cis variants Glu366Lys and Ile100Asn.
Chen S, DeMarco ML, Estey MP, Kyle B, Parker ML, Agbor TA, Kawada P, Speevak M, Nelson TN, Mattman A.
Clin Biochem. 2020 May;79:23-27. doi: 10.1016/j.clinbiochem.2020.02.013. Epub 2020 Feb 19.
PMID: 32087139

A Stress Buffering Perspective on the Progression of Alpha-1 Antitrypsin Deficiency.
Tian X, Solomon DH, Smith RA.
Health Commun. 2020 May;35(6):747-755. doi: 10.1080/10410236.2019.1584782. Epub 2019 Mar 7.
PMID: 30843437

Prevalence of Anti-Neutrophil Cytoplasmic Antibodies and Associated Vasculitis in COPD Associated With Alpha-1 Antitrypsin Deficiency: An Ancillary Study to a Prospective Study on 180 French Patients.
Deshayes S, Silva NM, Khoy K, Mariotte D, Le Mauff B, Mornex JF, Pison C, Cuvelier A, Balduyck M, Pujazon MC, Fournier M, Ilalne BA, Thabut G, Mal H, Aouba A.
Chest. 2020 May 14:S0012-3692(20)31405-7. doi: 10.1016/j.chest.2020.04.054. Online ahead of print.
PMID: 32417149 No abstract available.

Response to rituximab of pseudotumoral digital nodules in a patient with granulomatosis with polyangiitis with alpha-1 antitrypsin deficiency: A case report.
Aitmehdi R, Moguelet P, Godot S, Chazerain P, Senet P, Barbaud A, Frances C, Chasset F.
Dermatol Ther. 2020 May 2:e13479. doi: 10.1111/dth.13479. Online ahead of print.
PMID: 32359128 No abstract available.

Geographical Overlap Between Alpha-1 Antitrypsin Deficiency and COVID-19 Infection in Italy: Casual or Causal?
Vianello A, Braccioni F.
Arch Bronconeumol. 2020 May 31:S0300-2896(20)30169-1. doi: 10.1016/j.arbres.2020.05.015. Online ahead of print.
PMID: 32576394 Free PMC article. No abstract available.

Panniculitis as main clinical manifestation of alpha-1 antitrypsin deficiency revealing a SerpinA1 gene mutation.
Abdalla BMZ, Criado PR.
Australas J Dermatol. 2020 May 19. doi: 10.1111/ajd.13332. Online ahead of print.
PMID: 32430912 No abstract available.

April 2020 Publications

Alpha1-Antitrypsin Deficiency.
Strnad P, McElvaney NG, Lomas DA.
N Engl J Med. 2020 Apr 9;382(15):1443-1455. doi: 10.1056/NEJMra1910234.
PMID: 32268028 Review. No abstract available.

Activation of complement component 3 is associated with airways disease and pulmonary emphysema in alpha-1 antitrypsin deficiency.
O’Brien ME, Fee L, Browne N, Carroll TP, Meleady P, Henry M, McQuillan K, Murphy MP, Logan M, McCarthy C, McElvaney OJ, Reeves EP, McElvaney NG.
Thorax. 2020 Apr;75(4):321-330. doi: 10.1136/thoraxjnl-2019-214076. Epub 2020 Jan 20.
PMID: 31959730

Detection of alpha-1 antitrypsin deficiency: the past, present and future.
Brantly M, Campos M, Davis AM, D’Armiento J, Goodman K, Hanna K, O’Day M, Queenan J, Sandhaus R, Stoller J, Strange C, Teckman J, Wanner A.
Orphanet J Rare Dis. 2020 Apr 19;15(1):96. doi: 10.1186/s13023-020-01352-5.
PMID: 32306990 Free PMC article. Review.

α1 Antitrypsin therapy modulates the neutrophil membrane proteome and secretome.
Murphy MP, McEnery T, McQuillan K, McElvaney OF, McElvaney OJ, Landers S, Coleman O, Bussayajirapong A, Hawkins P, Henry M, Meleady P, Reeves EP, McElvaney NG.
Eur Respir J. 2020 Apr 30;55(4):1901678. doi: 10.1183/13993003.01678-2019. Print 2020 Apr.
PMID: 32060059

Evaluation of Alpha 1-Antitrypsin for the Early Diagnosis of Colorectal Cancer.
Jaberie H, Hosseini SV, Naghibalhossaini F.
Pathol Oncol Res. 2020 Apr;26(2):1165-1173. doi: 10.1007/s12253-019-00679-0. Epub 2019 Jun 10.
PMID: 31183614

Alpha-1 Antitrypsin Deficiency and Pregnancy.
Gaeckle NT, Stephenson L, Reilkoff RA.
COPD. 2020 Jun;17(3):326-332. doi: 10.1080/15412555.2020.1754778. Epub 2020 Apr 20.
PMID: 32308050

Systemic modified messenger RNA for replacement therapy in alpha 1-antitrypsin deficiency.
Karadagi A, Cavedon AG, Zemack H, Nowak G, Eybye ME, Zhu X, Guadagnin E, White RA, Rice LM, Frassetto AL, Strom S, Jorns C, Martini PGV, Ellis E.

Alpha 1 Antitrypsin Mutation.
Abdulkarim A, Craig TJ.
2020 Apr 17. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan–.
PMID: 29489299 Free Books & Documents. Review.

Engineering the serpin α1 -antitrypsin: A diversity of goals and techniques.
Scott BM, Sheffield WP.
Protein Sci. 2020 Apr;29(4):856-871. doi: 10.1002/pro.3794. Epub 2019 Dec 9.
PMID: 31774589 Review.

March 2020 Publications

Alpha-1-Antitrypsin Deficiency and Bronchiectasis: A Concomitance or a Real Association?
Sanduzzi A, Ciasullo E, Capitelli L, Sanduzzi Zamparelli S, Bocchino M.
Int J Environ Res Public Health. 2020 Mar 29;17(7). pii: E2294. doi: 10.3390/ijerph17072294.
PMID: 32235324 [PubMed – in process]

Proteomic and genomic biomarkers for Non-Small Cell Lung Cancer: Peroxiredoxin, Haptoglobin,
and Alpha-1 antitrypsin.
Najafi Z, Mohamadnia A, Ahmadi R, Mahmoudi M, Bahrami N, Khosravi A, Jamaati H, Tabarsi P,
Kazem Pour Dizaji M, Shirian S.
Cancer Med. 2020 Mar 30. doi: 10.1002/cam4.3019. [Epub ahead of print]
PMID: 32232956 [PubMed – as supplied by publisher]

A Novel Serine Protease Inhibitor PE-BBI Ameliorates Cockroach Extract-Mediated Airway
Epithelial Barrier Dysfunction.
Reihill JA, Ouyang X, Yang Z, Douglas LEJ, Zhou M, Chen T, Martin SL.
Biomolecules. 2020 Mar 28;10(4). pii: E515. doi: 10.3390/biom10040515.
PMID: 32231120 [PubMed – in process]

Identification of a novel alkaline serine protease from gazami crab (Portunus trituberculatus)
hepatopancreas and its hydrolysis of myofibrillar protein.
Song C, Shi Y, Meng X, Wu D, Zhang L.
Int J Biol Macromol. 2020 Mar 27;155:403-410. doi: 10.1016/j.ijbiomac.2020.03.179. [Epub ahead
of print] PMID: 32229212 [PubMed – as supplied by publisher]

Liver Involvement in Children with Alpha-1 Antitrypsin Deficiency: A Multicenter Study.
Cakir M, Sag E, Islek A, Baran M, Tumgor G, Aydogdu S.
Pediatr Gastroenterol Hepatol Nutr. 2020 Mar;23(2):146-153. doi: 10.5223/pghn.2020.23.2.146.
Epub 2020 Mar 4.
PMID: 32206627 [PubMed]

A serine protease inhibitor suppresses autoimmune neuroinflammation by activating the STING/IFNβ axis in macrophages.
Casella G, Rasouli J, Mason K, Boehm A, Kumar G, Hwang D, Thome R, Ishikawa L, Zhang GX,
Ciric B, Rostami A.
Cell Mol Immunol. 2020 Mar 20. doi: 10.1038/s41423-020-0405-z. [Epub ahead of print] No abstract
available. PMID: 32203194 [PubMed – as supplied by publisher]

Clinical considerations in individuals with Alpha-1 Antitrypsin PI*SZ genotype.
McElvaney GN, Sandhaus RA, Miravitlles M, Turino GM, Seersholm N, Wencker M, Stockley RA.
Eur Respir J. 2020 Mar 12. pii: 1902410. doi: 10.1183/13993003.02410-2019. [Epub ahead of print]
Review.

Beyond hemostasis: a snake venom serine protease with potassium channel blocking and potential
antitumor activities.
Boldrini-França J, Pinheiro-Junior EL, Peigneur S, Pucca MB, Cerni FA, Borges RJ, Costa TR,
Carone SEI, Fontes MRM, Sampaio SV, Arantes EC, Tytgat J.
Sci Rep. 2020 Mar 11;10(1):4476. doi: 10.1038/s41598-020-61258-x.
PMID: 32161292 [PubMed – in process]

Studies of Thioamide Effects on Serine Protease Activity Enable Two-Site Stabilization of Cancer
Imaging Peptides.
Barrett TM, Chen XS, Liu C, Giannakoulias S, Phan HAT, Wang J, Keenan EK, Karpowicz RJ Jr,
Petersson EJ.
ACS Chem Biol. 2020 Mar 20;15(3):774-779. doi: 10.1021/acschembio.9b01036. Epub 2020 Mar 6.
PMID: 32141733 [PubMed – in process]

Corrigendum to “Comparison of mammalian and bacterial expression library screening to detect
recombinant alpha-1 proteinase inhibitor variants with enhanced thrombin inhibitory capacity” [J.
Biotechnol. 208 (2015) 54-62].
Gierczak RF, Bhakta V, Xie M, Sheffield WP.
J Biotechnol. 2020 Mar 10;311:59. doi: 10.1016/j.jbiotec.2020.02.013. Epub 2020 Mar 2. No
abstract available.
PMID: 32139085 [PubMed]

Alpha-1 antitrypsin inhibits formaldehyde-induced apoptosis of human peritoneal mesothelial cells.
Lim JH, Park SM, Yook JM, Ahn JS, Choi SY, Oh SH, Jung HY, Choi JY, Cho JH, Park SH, Kim
YL, Kim CD.
Perit Dial Int. 2020 Mar;40(2):124-131. doi: 10.1177/0896860819887288. Epub 2020 Jan 17.
PMID: 32063193 [PubMed – in process]

Diagnosis and treatment of lung disease associated with alpha one-antitrypsin deficiency: A position
statement from the Thoracic Society of Australia and New Zealand.
Dummer J, Dobler CC, Holmes M, Chambers D, Yang IA, Parkin L, Smith S, Wark P, Dev A,
Hodge S, Dabscheck E, Gooi J, Samuel S, Knowles S, Holland AE.
Respirology. 2020 Mar;25(3):321-335. doi: 10.1111/resp.13774. Epub 2020 Feb 6. Review.
PMID: 32030868 [PubMed – in process

α-1 Antitrypsin as a potential biomarker in chronic heart failure.
Lubrano V, Vergaro G, Maltinti M, Ghionzoli N, Emdin M, Papa A.
J Cardiovasc Med (Hagerstown). 2020 Mar;21(3):209-215. doi: 10.2459/JCM.0000000000000937.
PMID: 32004242 [PubMed – in process]

A new serine protease family with elastase activity is produced by Streptomyces bacteria.
Fujii T, Fukano K, Hirano K, Mimura A, Terauchi M, Etoh SI, Iida A.
Microbiology. 2020 Mar;166(3):253-261. doi: 10.1099/mic.0.000880.
PMID: 31896394 [PubMed – in process]

Alpha-1 Antitrypsin Mutations: Is One Too Many?
Sandford AJ.
Am J Respir Crit Care Med. 2020 Mar 1;201(5):505-506. doi: 10.1164/rccm.201911-2209ED. No
abstract available.
PMID: 31810377 [PubMed – in process]

The Effects of Rare SERPINA1 Variants on Lung Function and Emphysema in SPIROMICS.
Ortega VE, Li X, O’Neal WK, Lackey L, Ampleford E, Hawkins GA, Grayeski PJ, Laederach A,
Barjaktarevic I, Barr RG, Cooper C, Couper D, Han MK, Kanner RE, Kleerup EC, Martinez FJ,
Paine R 3rd, Peters SP, Pirozzi C, Rennard SI, Woodruff PG, Hoffman EA, Meyers DA, Bleecker
ER; NHLBI Subpopulations and Intermediate Outcomes Measures in COPD Study (SPIROMICS).
Am J Respir Crit Care Med. 2020 Mar 1;201(5):540-554. doi: 10.1164/rccm.201904-0769OC.
PMID: 31661293 [PubMed – in process]

Anti-dengue infectivity evaluation of bioflavonoid from Azadirachta indica by dengue virus serine protease inhibition.
Dwivedi VD, Bharadwaj S, Afroz S, Khan N, Ansari MA, Yadava U, Tripathi RC, Tripathi IP,
Mishra SK, Kang SG.
J Biomol Struct Dyn. 2020 Mar 9:1-14. doi: 10.1080/07391102.2020.1734485. [Epub ahead of print]
PMID: 32107969 [PubMed – as supplied by publisher]

July 2019 Publications

Crystal structure of the inhibitor-free form of the serine protease kallikrein-4.
Riley BT, Hoke DE, McGowan S, Buckle AM.
Acta Crystallogr F Struct Biol Commun. 2019 Aug 1;75(Pt 8):543-546. doi: 10.1107/S2053230X19009610. Epub 2019 Jul 16.
PMID: 31397325 [PubMed – in process]

Severe bilateral descemetoceles in Alpha-1 antitrypsin deficiency.
Ma J, Baqai J.
Am J Ophthalmol Case Rep. 2019 Jul 10;15:100513. doi: 10.1016/j.ajoc.2019.100513. eCollection 2019 Sep.
PMID: 31360795 [PubMed] Free PMC Article

Serine protease inhibitors and activators from Dalbergia tonkinensis species.
Son NT, Suenaga M, Matsunaga Y, Van Chinh L, Kubo M, Harada K, Cuong NM, Fukuyama Y.
J Nat Med. 2019 Jul 27. doi: 10.1007/s11418-019-01347-y. [Epub ahead of print]
PMID: 31352545 [PubMed – as supplied by publisher]

Alpha 1-antitrypsin for treating ventilator-associated lung injury in acute respiratory distress syndrome rats.
Wang X, Gong J, Zhu J, Jin Z, Gao W.
Exp Lung Res. 2019 Sep;45(7):209-219. doi: 10.1080/01902148.2019.1642968. Epub 2019 Jul 26.
PMID: 31347410 [PubMed – in process]

This list of publications was generated on PubMed using the following Keywords:

alpha 1-Antitrypsin
alpha one antitrypsin
alpha 1 antitrypsine
alpha1-AT deficiency
alpha-1-AT
alpha 1 antiproteinase
alpha 1 antiprotease
alpha 1 protease
alpha 1 proteinase
familial emphysema
genetic emphysema
AATD
A1AT
hA1AT
A1-AT
A1P1
Prolastin
Aralast
Zemaira
Glassia

Accreditations

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