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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. Previous months’ listings are archived in the left sidebar of this page.

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

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

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

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 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 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.

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.

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

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

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.

[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.

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: 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

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.

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

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.

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

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.

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.

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]

Antithrombin plus alpha-1 protease inhibitor does not affect coagulation and inflammation in two murine models of acute lung injury.
Juschten J, Ingelse SA, Maas MAW, Girbes ARJ, Juffermans NP, Schultz MJ, Tuinman PR.
Intensive Care Med Exp. 2019 Jul 25;7(Suppl 1):36. doi: 10.1186/s40635-019-0240-7.
PMID: 31346884 [PubMed] Free PMC Article

What Do Alpha-1 Antitrypsin Levels Tell Us About Chronic Inflammation in COPD?
Ellis P, Turner A.
Arch Bronconeumol. 2019 Jul 21. pii: S0300-2896(19)30285-6. doi: 10.1016/j.arbres.2019.06.010. [Epub ahead of print] English, Spanish. No abstract available.
PMID: 31340890 [PubMed – as supplied by publisher] Free Article

Characterization of active anticoagulant fraction and a fibrin(ogen)olytic serine protease from leaves of Clerodendrum colebrookianum, a traditional ethno-medicinal plant used to reduce hypertension.
Gogoi D, Ramani S, Bhartari S, Chattopadhyay P, Mukherjee AK.
J Ethnopharmacol. 2019 Oct 28;243:112099. doi: 10.1016/j.jep.2019.112099. Epub 2019 Jul 18.
PMID: 31326559 [PubMed – in process]

Vascular Alpha-1 Adrenergic Sensitivity is Enhanced in Chronic Kidney Disease.
Sprick JD, Morison DL, Stein CM, Li Y, Paranjape SY, Fonkoue IT, DaCosta DR, Park J.
Am J Physiol Regul Integr Comp Physiol. 2019 Jul 17. doi: 10.1152/ajpregu.00090.2019. [Epub ahead of print]
PMID: 31314543 [PubMed – as supplied by publisher]

Bioactive Thymosin Alpha-1 Does Not Influence F508del-CFTR Maturation and Activity.
Armirotti A, Tomati V, Matthes E, Veit G, Cholon DM, Phuan PW, Braccia C, Guidone D, Gentzsch M, Lukacs GL, Verkman AS, Galietta LJV, Hanrahan JW, Pedemonte N.
Sci Rep. 2019 Jul 16;9(1):10310. doi: 10.1038/s41598-019-46639-1.
PMID: 31311979 [PubMed – in process] Free PMC Article

Protein modeling to assess the pathogenicity of rare variants of SERPINA1 in patients suspected of having Alpha 1 Antitrypsin Deficiency.
Kueppers F, Andrake MD, Xu Q, Dunbrack RL Jr, Kim J, Sanders CL.
BMC Med Genet. 2019 Jul 15;20(1):125. doi: 10.1186/s12881-019-0852-5.
PMID: 31307431 [PubMed – in process] Free PMC Article

Metacaspase-3 of Plasmodium falciparum: An atypical trypsin-like serine protease.
Kumar B, Verma S, Kashif M, Sharma R, Atul, Dixit R, Singh AP, Pande V, Saxena AK, Abid M, Pandey KC.
Int J Biol Macromol. 2019 Jul 10;138:309-320. doi: 10.1016/j.ijbiomac.2019.07.067. [Epub ahead of print]
PMID: 31301397 [PubMed – as supplied by publisher]

SERPINA1 gene polymorphisms in a population-based ALSPAC cohort.
DeLuca DS, Poluzioroviene E, Taminskiene V, Wrenger S, Utkus A, Valiulis A, Alasevičius T, Henderson J, Bush A, Welte T, Janciauskiene S, Valiulis A.
Pediatr Pulmonol. 2019 Jul 12. doi: 10.1002/ppul.24422. [Epub ahead of print]
PMID: 31298815 [PubMed – as supplied by publisher]

Down-regulation of tissue levels of serine protease inhibitor (vaspin) in psoriasis vulgaris patients: a possible mechanism of narrowband ultraviolet B radiation.
Sayed KS, Said ER, Mohammed FN, Algorhary M, Rashed LA.
Arch Dermatol Res. 2019 Jul 10. doi: 10.1007/s00403-019-01947-4. [Epub ahead of print]
PMID: 31292731 [PubMed – as supplied by publisher]

para-Sulphonato-calix[n]arene capped silver nanoparticles challenge the catalytic efficiency and the stability of a novel human gut serine protease inhibitor.
Akermi N, Mkaouar H, Kriaa A, Jablaoui A, Soussou S, Gargouri A, Coleman AW, Perret F, Maguin E, Rhimi M.
Chem Commun (Camb). 2019 Aug 7;55(61):8935-8938. doi: 10.1039/c9cc03183a. Epub 2019 Jul 9.
PMID: 31286126 [PubMed – indexed for MEDLINE]

Light scattering determination of the stoichiometry for protease-potato serine protease inhibitor complexes.
Billinger E, Zuo S, Lundmark K, Johansson G.
Anal Biochem. 2019 Oct 1;582:113357. doi: 10.1016/j.ab.2019.113357. Epub 2019 Jul 2.
PMID: 31276650 [PubMed – in process]

Alpha-1 Antitrypsin Replacement in Patients With COPD.
Wells AD, Woods A, Hilleman DE, Malesker MA.
P T. 2019 Jul;44(7):412-415.
PMID: 31258312 [PubMed] Free PMC Article

Alpha-1 Antitrypsin Deficiency: A Predisposing Factor for the Development of Pulmonary Langerhans Cell Histiocytosis.
Ellis PR, Campbell EJ, Turner AM, Stockley RA.
Chronic Obstr Pulm Dis. 2019 Jul 24;6(3):206-209. doi: 10.15326/jcopdf.6.3.2019.0129. No abstract available.
PMID: 31247686 [PubMed] Free Article

The reaction mechanism of Zika virus NS2B/NS3 serine protease inhibition by dipeptidyl aldehyde: a QM/MM study.
Nutho B, Mulholland AJ, Rungrotmongkol T.
Phys Chem Chem Phys. 2019 Jul 10;21(27):14945-14956. doi: 10.1039/c9cp02377a.
PMID: 31236554 [PubMed – indexed for MEDLINE]

The α-1,6-mannosyltransferase VdOCH1 plays a major role in microsclerotium formation and virulence in the soil-borne pathogen Verticillium dahliae.
Zhang J, Zhang Y, Yang J, Kang L, EloRM AM, Zhou H, Zhao J.
Fungal Biol. 2019 Jul;123(7):539-546. doi: 10.1016/j.funbio.2019.05.007. Epub 2019 May 18.
PMID: 31196523 [PubMed – in process]

Proinflammatory Effects of Cardiotonic Steroids Mediated by NKA α-1 (Na+/K+-ATPase α-1)/Src Complex in Renal Epithelial Cells and Immune Cells.
Khalaf FK, Dube P, Kleinhenz AL, Malhotra D, Gohara A, Drummond CA, Tian J, Haller ST, Xie Z, Kennedy DJ.
Hypertension. 2019 Jul;74(1):73-82. doi: 10.1161/HYPERTENSIONAHA.118.12605. Epub 2019 May 28.
PMID: 31132948 [PubMed – in process]

The prognostic potential of alpha-1 type I collagen expression in papillary thyroid cancer.
Huang C, Yang X, Han L, Fan Z, Liu B, Zhang C, Lu T.
Biochem Biophys Res Commun. 2019 Jul 12;515(1):125-132. doi: 10.1016/j.bbrc.2019.04.119. Epub 2019 May 22.
PMID: 31128912 [PubMed – in process]

Targeting the site encoded by SERPINA1*E342K for treating alpha-1 antitrypsin deficiency-associated liver diseases.
Zhang X, Pham K, Li D, Schutte RJ, Brantly M, Liu C, Ostrov DA.
FEBS Lett. 2019 Jul;593(14):1849-1862. doi: 10.1002/1873-3468.13452. Epub 2019 Jun 6.
PMID: 31116417 [PubMed – in process]

In vitro silencing of a serine protease inhibitor suppresses Trichinella spiralis invasion, development, and fecundity.
Yang F, Yang DQ, Song YY, Guo KX, Li YL, Long SR, Jiang P, Cui J, Wang ZQ.
Parasitol Res. 2019 Jul;118(7):2247-2255. doi: 10.1007/s00436-019-06344-4. Epub 2019 May 12.
PMID: 31081529 [PubMed – in process]

Liver Disease Due to Alpha-1 Antitrypsin Deficiency: Are We Surprised That It Is More Complex Than We Thought?
Di Bisceglie AM, Teckman J.
Hepatology. 2019 Jul;70(1):5-7. doi: 10.1002/hep.30694. Epub 2019 May 31. No abstract available.
PMID: 31058323 [PubMed – in process]

GDNF family receptor alpha 1 is a reliable marker of undifferentiated germ cells in bulls.
Kim YH, Choi YR, Kim BJ, Jung SE, Kim SM, Jin JH, Yun MH, Kim SU, Kim YH, Hwang S, Pang MG, Ryu BY.
Theriogenology. 2019 Jul 1;132:172-181. doi: 10.1016/j.theriogenology.2019.04.016. Epub 2019 Apr 16.
PMID: 31029848 [PubMed – in process]

Immunoreactivity changes of human serum albumin and alpha-1-microglobulin induced by their interaction with dendrimers.
Serchenya T, Shcharbin D, Shyrochyna I, Sviridov O, Terekhova M, Dzmitruk V, Abashkin V, Apartsin E, Mignani S, Majoral JP, Ionov M, Bryszewska M.
Colloids Surf B Biointerfaces. 2019 Jul 1;179:226-232. doi: 10.1016/j.colsurfb.2019.03.065. Epub 2019 Apr 4.
PMID: 30974260 [PubMed – in process]

The Plasmodium berghei serine protease PbSUB1 plays an important role in male gamete egress.
Pace T, Grasso F, Camarda G, Suarez C, Blackman MJ, Ponzi M, Olivieri A.
Cell Microbiol. 2019 Jul;21(7):e13028. doi: 10.1111/cmi.13028. Epub 2019 Apr 29.
PMID: 30941868 [PubMed – in process] Free Article

How many protein molecules are secreted by single Helicobacter pylori cells: Quantification of serine protease HtrA.
Neddermann M, Backert S.
Cell Microbiol. 2019 Jul;21(7):e13022. doi: 10.1111/cmi.13022. Epub 2019 Mar 19.
PMID: 30822363 [PubMed – in process]

Tissue and Exosomal Serine Protease Inhibitors Are Significantly Overexpressed in Chronic Rhinosinusitis With Nasal Polyps.
Mueller SK, Nocera AL, Dillon ST, Libermann TA, Wendler O, Bleier BS.
Am J Rhinol Allergy. 2019 Jul;33(4):359-368. doi: 10.1177/1945892419831108. Epub 2019 Feb 27. No abstract available.
PMID: 30810048 [PubMed – in process]

Alpha-1 Antitrypsin Deficiency Liver Disease, Mutational Homogeneity Modulated by Epigenetic Heterogeneity With Links to Obesity.
Wang L, Marek GW 3rd, Hlady RA, Wagner RT, Zhao X, Clark VC, Fan AX, Liu C, Brantly M, Robertson KD.
Hepatology. 2019 Jul;70(1):51-66. doi: 10.1002/hep.30526. Epub 2019 Mar 20.
PMID: 30681738 [PubMed – in process]

Increased Active OMI/HTRA2 Serine Protease Displays a Positive Correlation with Cholinergic Alterations in the Alzheimer’s Disease Brain.
Darreh-Shori T, Rezaeianyazdi S, Lana E, Mitra S, Gellerbring A, Karami A, Bogdanovic N, Lithner CU, Winblad B, Behbahani H.
Mol Neurobiol. 2019 Jul;56(7):4601-4619. doi: 10.1007/s12035-018-1383-3. Epub 2018 Oct 25.
PMID: 30361890 [PubMed – in process] Free PMC Article

Alpha-1 Adrenergic Receptor Agonist Phenylephrine Inhibits Sepsis-Induced Cardiomyocyte Apoptosis and Cardiac Dysfunction via Activating ERK1/2 Signal Pathway.
Li H, Xing Y, Yang D, Tang X, Lu D, Wang H.
Shock. 2019 Jul;52(1):122-133. doi: 10.1097/SHK.0000000000001205. PMID: 29889817 [PubMed – in process]

  • alpha 1-Antitrypsin
  • alpha one antitrypsin
  • alpha 1 antitrypsine
  • alpha1-AT deficiency
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  • alpha 1 antiproteinase
  • alpha 1 antiprotease
  • alpha 1 protease
  • alpha 1 proteinase
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  • genetic emphysema
  • AATD
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  • hA1AT
  • A1-AT
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