Literature for inhibitor I04.001: alpha-1-peptidase inhibitor

Summary Gene structure Alignment Tree Sequences Sequence features Distribution Structure Literature

(Topics flags: S Structure, P Specificity, K Knockout, E Expression, V Review, M Mutation, I Inhibitor, U Therapeutic. To select only the references relevant to a single topic, click the link above. See explanation.)

2026

Mawani,R., Pye,A. and Turner,A.M. Alpha-1 Antitrypsin Deficiency: Current Landscape of Detection, Management, and Treatment Adv Ther (2026) PubMed Europe PubMed DOI
Yamauchi,T., Luo,Y., Menon,D.R., Couts,K., Khan,S., Goel,A., Dinarello,C.A., Zhai,Z. and Fujita,M. Human Alpha-1 Antitrypsin Suppresses Melanoma Growth by Promoting Tumor Differentiation and CD8(+) T-Cell-Mediated Immunity Biomolecules (2026) 16 PubMed Europe PubMed DOI PMC EPMC

2025

Abdulkarim,A. and Craig,T.J. Alpha 1 Antitrypsin Mutation (2025) PubMed Europe PubMed
Bhakta,V., Tehrani,N.C., Ask,A. and Sheffield,W.P. Expression and purification of a broad-spectrum human protease inhibitor in Pichia pastoris Biochem Biophys Rep (2025) 43, 102092-102092. PubMed Europe PubMed DOI PMC EPMC
Boyle,L.D., Miguelez-Crespo,A., Paul,M., Villalobos,E., Toews,J.N.C., Ivatt,L., Nagy,B., Magennis,M., Homer,N.Z.M., Andrew,R., Viau,V., Hammond,G.L., Stimson,R.H., Walker,B.R. and Nixon,M. The NE/AAT/CBG axis regulates adipose tissue glucocorticoid exposure Nat Commun (2025) 16, 545-545. PubMed Europe PubMed DOI PMC EPMC
Cheng,S.Y., Giguere,D., Silverstein,I., Conza,A., Seddon,J.M., Kim,S., Iwata,T., Mueller,C. and Punzo,C. Role of alpha-1 antitrypsin in Bruch's membrane integrity Sci Rep (2025) 15, 12223-12223. PubMed Europe PubMed DOI PMC EPMC
Elshikha,A.S., Abboud,G., Avdiaj,R., Morel,L. and Song,S. The Inhibitory Effects of Alpha 1 Antitrypsin on Endosomal TLR Signaling Pathways Biomolecules (2025) 15 PubMed Europe PubMed DOI PMC EPMC
Engelmaier,A., Zimmermann,M., Butterweck,H.A. and Weber,A. Clinical Study Support by Long-Term Stability Studies of Alpha(1)-Proteinase Inhibitor and Urea in Relevant Biological Matrices Pharmaceuticals (Basel) (2025) 18 PubMed Europe PubMed DOI PMC EPMC
Farber,I., Sheffer,L., Anav,Y., Goldstein,O., Halpern,D., Pinto,N., Cohen,S., Darawsha,A., Ogen-Shtern,N., El-Saied,S., Lewis,E.C. and Silberstein,E. Oxidation-dependent effects of alpha-1 antitrypsin on wound healing and inflammation Sci Rep (2025) 15, 40673-40673. PubMed Europe PubMed DOI PMC EPMC
Fromme,M., Klebingat,F., Ellis,P. and Strnad,P. Alpha-1 antitrypsin deficiency-associated liver disease: From understudied disorder to the poster child of genetic medicine Hepatol Commun (2025) 9 PubMed Europe PubMed DOI PMC EPMC V
Gordon,M., Gangemi,A.J., Sandwith,E.L., Kumaran,M. and Kueppers,F. Vascular Pathology in Alpha 1 Antitrypsin Deficient Chronic Obstructive Pulmonary Disease and Emphysema Patients: Case Reports Appl Clin Genet (2025) 18, 1-7. PubMed Europe PubMed DOI PMC EPMC
Huntington,J.A., Reckless,J., Polderdijk,S.G.I., Hextall,P. and Baglin,T. The preclinical profile of SerpinPC- a potential new treatment for hemophilia Blood Adv (2025) PubMed Europe PubMed DOI
Jiamutai,F.N.U., Hatfield,A., Herbert,A., Majumdar,D., Shankar,V. and Lackey,L. Altered polyadenylation site usage in SERPINA1 3'UTR in response to cellular stress affects A1AT protein expression Sci Rep (2025) 15, 23510-23510. PubMed Europe PubMed DOI PMC EPMC
Rajab,I., Marin,M.P., Shubietah,A., Khaled,A., Bdair,M., Abukhalil,M.M., Jallad,H., Hussein,A., Aldwaik,S.A. and Salvatore,M.M. Alpha-1 antitrypsin deficiency: genetics, clinical manifestations, AI prognostics, and advanced imaging in liver disease Ann Med Surg (Lond) (2025) 87, 7303-7318. PubMed Europe PubMed DOI PMC EPMC
Sayed,H., Mayo,K.H., Zhou,Y., Tai,G. and Su,J. Galectin-8 binding to alpha-1 antitrypsin is a physiological mechanism in healthy individuals but exacerbates the symptoms of alpha-1 antitrypsin deficiency Arch Biochem Biophys (2025) 772, 110577-110577. PubMed Europe PubMed DOI
Wang,T., Shuai,P., Wang,Q., Guo,C., Huang,S., Li,Y., Wu,W. and Yi,L. alphaƒ_'1 Antitrypsin is a potential target of inflammation and immunomodulation (Review) Mol Med Rep (2025) 31 PubMed Europe PubMed DOI PMC EPMC
Xiang,S., Yang,L., He,Y., Ding,F., Qiao,S., Su,Z., Chen,Z., Lu,A. and Li,F. Alpha-1 Antitrypsin as a Regulatory Protease Inhibitor Modulating Inflammation and Shaping the Tumor Microenvironment in Cancer Cells (2025) 14 PubMed Europe PubMed DOI PMC EPMC V
Zhang,L., Bai,X., Du,M., Dou,W., Xie,Z., Liu,J. and Hou,Y. SERPINA1 drives TACE resistance in hepatocellular carcinoma by competitively binding ITGB3 to block ITCH-mediated ubiquitination and degradation Cell Oncol (Dordr) (2025) 49, 11-11. PubMed Europe PubMed DOI PMC EPMC

2024

Bai,X., Gao,J., Guan,X., Narum,D.E., Fornis,L.B., Griffith,D.E., Gao,B., Sandhaus,R.A., Huang,H. and Chan,E.D. Analysis of alpha-1-antitrypsin (AAT)-regulated, glucocorticoid receptor-dependent genes in macrophages reveals a novel host defense function of AAT Physiol Rep (2024) 12, e16124-e16124. PubMed Europe PubMed DOI PMC EPMC
Balduyck,M., Afif,S., Onraed,B., Jourdain,M., Nseir,S., Pigny,P. and Zerimech,F. Does hypersialylation compensate the functional Alpha1-AntiTrypsin (A1AT) deficiency in all critically ill patients? Biochimie (2024) PubMed Europe PubMed DOI
Chen,C.H., Crisford,H., Scott,A., Sapey,E. and Stockley,R.A. A novel in vitro cell model of the proteinase/antiproteinase balance observed in alpha-1 antitrypsin deficiency Front Pharmacol (2024) 15, 1421598-1421598. PubMed Europe PubMed DOI PMC EPMC
Fahndrich,S. and Bals,R. [Alpha 1-antitrypsin deficiency] Inn Med (Heidelb) (2024) 65, 533-537. PubMed Europe PubMed DOI
Kontoh-Twumasi,R., Budkin,S., Edupuganti,N., Vashishtha,A. and Sharma,S. Role of Serine Protease Inhibitors A1 and A3 in Ocular Pathologies Invest Ophthalmol Vis Sci (2024) 65, 16-16. PubMed Europe PubMed DOI PMC EPMC V
Lietz,S., Sokolowski,L.M., Barth,H. and Ernst,K. Alpha-1 antitrypsin inhibits Clostridium botulinum C2 toxin, Corynebacterium diphtheriae diphtheria toxin and B. anthracis fusion toxin Sci Rep (2024) 14, 21257-21257. PubMed Europe PubMed DOI PMC EPMC
Martinelli,I., Zucchi,E., Simonini,C., Gianferrari,G., Bedin,R., Biral,C., Ghezzi,A., Fini,N., Carra,S. and Mandrioli,J. SerpinA1 levels in amyotrophic lateral sclerosis patients: An exploratory study Eur J Neurol (2024) 31, e16054-e16054. PubMed Europe PubMed DOI
Mohammad,N., Oshins,R., Gu,T., Clark,V., Lascano,J., Assarzadegan,N., Marek,G., Brantly,M. and Khodayari,N. Liver Characterization of a Cohort of Alpha-1 Antitrypsin Deficiency Patients with and without Lung Disease J Clin Transl Hepatol (2024) 12, 845-856. PubMed Europe PubMed DOI PMC EPMC
Mulkareddy,V. and Roman,J. Pulmonary manifestations of alpha 1 antitrypsin deficiency Am J Med Sci (2024) 368, 1-8. PubMed Europe PubMed DOI V
Okagawa,S., Sakaguchi,M., Okubo,Y., Takekuma,Y., Igata,M., Kondo,T., Takeda,N., Araki,K., Brandao,B.B., Qian,W.J., Tseng,Y.H., Kulkarni,R.N., Kubota,N., Kahn,C.R. and Araki,E. Hepatic SerpinA1 improves energy and glucose metabolism through regulation of preadipocyte proliferation and UCP1 expression Nat Commun (2024) 15, 9585-9585. PubMed Europe PubMed DOI PMC EPMC
Park,Y., Matsumoto,S., Ogata,K., Ma,B., Kanada,R., Isaka,Y., Arichi,N., Liang,X., Maki,R., Kozasa,T., Okuno,Y., Ohno,H., Ishihama,Y. and Toyoshima,F. Receptor-independent regulation of Galpha13 by alpha-1-antitrypsin C-terminal peptides J Biol Chem (2024) , 108136-108136. PubMed Europe PubMed DOI
Xiubing,C., Huazhen,L., Xueyan,W., Jing,N., Qing,L., Haixing,J., Shanyu,Q. and Jiefu,L. SERPINA1 promotes the invasion, metastasis, and proliferation of pancreatic ductal adenocarcinoma via the PI3K/Akt/NF-kappaB pathway Biochem Pharmacol (2024) 230, 116580-116580. PubMed Europe PubMed DOI K

2023

Bai,X., Schountz,T., Buckle,A.M., Talbert,J.L., Sandhaus,R.A. and Chan,E.D. Alpha-1-antitrypsin antagonizes COVID-19: a review of the epidemiology, molecular mechanisms, and clinical evidence Biochem Soc Trans (2023) 51, 1361-1375. PubMed Europe PubMed DOI PMC EPMC
Kaur,U., Kihn,K.C., Ke,H., Kuo,W., Gierasch,L.M., Hebert,D.N., Wintrode,P.L., Deredge,D. and Gershenson,A. The conformational landscape of a serpin N-terminal subdomain facilitates folding and in-cell quality control bioRxiv (2023) PubMed Europe PubMed DOI PMC EPMC
Khoshdel,A., Forootan,M., Afsharinasab,M., Rezaian,M. and Abbasifard,M. Assessment of the circulatory concentrations of cathepsin D, cathepsin K, and alpha-1 antitrypsin in patients with knee osteoarthritis Ir J Med Sci (2023) 192, 1191-1196. PubMed Europe PubMed DOI
Wiesemann,G.S., Oshins,R.A., Flagg,T.O. and Brantly,M.L. Novel SERPINA1 Alleles Identified through a Large Alpha-1 Antitrypsin Deficiency Screening Program and Review of Known Variants Chronic Obstr Pulm Dis (2023) 10, 7-21. PubMed Europe PubMed DOI PMC EPMC V
Wu,T., Hagiwara,M., Gnass,E., Barman,H., Sasson,D., Treem,W., Ren,K., Marins,E.G., Karki,C. and Malhi,H. Liver disease progression in patients with alpha-1 antitrypsin deficiency and protease inhibitor ZZ genotype with or without lung disease Aliment Pharmacol Ther (2023) 58, 1075-1085. PubMed Europe PubMed DOI

2022

Akbasheva,O.E., Spirina,L.V., Dyakov,D.A. and Masunova,N.V. Proteolysis and Deficiency of alpha1-Proteinase Inhibitor in SARS-CoV-2 Infection Biochem Mosc Suppl B Biomed Chem (2022) 16, 271-291. PubMed Europe PubMed DOI PMC EPMC V
Akbasheva,O.E., Spirina,L.V., Dyakov,D.A. and Masunova,N.V. [Proteolysis and deficiency of alpha1-proteinase inhibitor in SARS-CoV-2 infection] Biomed Khim (2022) 68, 157-176. PubMed Europe PubMed DOI V
D'Amato,M., Vertui,V., Pandolfi,L., Bozzini,S., Fossali,T., Colombo,R., Aliberti,A., Fumagalli,M., Iadarola,P., Dido,C., Viglio,S. and Meloni,F. Investigating the Link between Alpha-1 Antitrypsin and Human Neutrophil Elastase in Bronchoalveolar Lavage Fluid of COVID-19 Patients Curr Issues Mol Biol (2022) 44, 2122-2138. PubMed Europe PubMed DOI PMC EPMC
Grayeski,P.J., Weidmann,C.A., Kumar,J., Lackey,L., Mustoe,A.M., Busan,S., Laederach,A. and Weeks,K.M. Global 5'-UTR RNA structure regulates translation of a SERPINA1 mRNA Nucleic Acids Res (2022) 50, 9689-9704. PubMed Europe PubMed DOI PMC EPMC
Liu,W., Du,M., Wan,H., Yang,H., Deng,X., Chen,Y. and Zhang,Q. Serpin family A member 1 is an oncogene in glioma and its translation is enhanced by NAD(P)H quinone dehydrogenase 1 through RNA-binding activity Open Med (Wars) (2022) 17, 1645-1654. PubMed Europe PubMed DOI PMC EPMC
Liu,X., Kouassi,K.G.W., Vanbever,R. and Dumoulin,M. Impact of the PEG length and PEGylation site on the structural, thermodynamic, thermal, and proteolytic stability of mono-PEGylated alpha-1 antitrypsin Protein Sci (2022) 31, e4392-e4392. PubMed Europe PubMed DOI PMC EPMC
Miravitlles,M., Turner,A.M., Torres-Duran,M., Tanash,H., Rodriguez-Garcia,C., Lopez-Campos,J.L., Chlumsky,J., Guimaraes,C., Rodriguez-Hermosa,J.L., Corsico,A., Martinez-Gonzalez,C., Hernandez-Perez,J.M., Bustamante,A., Parr,D.G., Casas-Maldonado,F., Hecimovic,A., Janssens,W., Lara,B., Barrecheguren,M., Gonzalez,C., Stolk,J., Esquinas,C. and Clarenbach,C.F. Clinical and functional characteristics of individuals with alpha-1 antitrypsin deficiency: EARCO international registry Respir Res (2022) 23, 352-352. PubMed Europe PubMed DOI PMC EPMC
O'Brien,M.E., Murray,G., Gogoi,D., Yusuf,A., McCarthy,C., Wormald,M.R., Casey,M., Gabillard-Lefort,C., McElvaney,N.G. and Reeves,E.P. A Review of Alpha-1 Antitrypsin Binding Partners for Immune Regulation and Potential Therapeutic Application Int J Mol Sci (2022) 23 PubMed Europe PubMed DOI PMC EPMC V
Pini,L., Giordani,J., Ciarfaglia,M., Pini,A., Arici,M. and Tantucci,C. Alpha1-antitrypsin deficiency and cardiovascular disease: questions and issues of a debated relation J Cardiovasc Med (Hagerstown) (2022) 23, 637-645. PubMed Europe PubMed DOI V
Strnad,P., Mandorfer,M., Choudhury,G., Griffiths,W., Trautwein,C., Loomba,R., Schluep,T., Chang,T., Yi,M., Given,B.D., Hamilton,J.C., San Martin,J. and Teckman,J.H. Fazirsiran for Liver Disease Associated with Alpha1-Antitrypsin Deficiency N Engl J Med (2022) 387, 514-524. PubMed Europe PubMed DOI
Sun,R., Xu,Z., Zhu,C., Chen,T., Munoz,L.E., Dai,L. and Zhao,Y. Alpha-1 antitrypsin in autoimmune diseases: Roles and therapeutic prospects Int Immunopharmacol (2022) 110, 109001-109001. PubMed Europe PubMed DOI V
Tiensuu,H., Haapalainen,A.M., Tissarinen,P., Pasanen,A., Maatta,T.A., Huusko,J.M., Ohlmeier,S., Bergmann,U., Ojaniemi,M., Muglia,L.J., Hallman,M. and Ramet,M. Human placental proteomics and exon variant studies link AAT/SERPINA1 with spontaneous preterm birth BMC Med (2022) 20, 141-141. PubMed Europe PubMed DOI PMC EPMC K
Wilkinson,D.J., Falconer,A.M.D., Wright,H.L., Lin,H., Yamamoto,K., Cheung,K., Charlton,S.H., Arques,M.D.C., Janciauskiene,S., Refaie,R., Rankin,K.S., Young,D.A. and Rowan,A.D. Matrix metalloproteinase-13 is fully activated by neutrophil elastase and inactivates its serpin inhibitor, alpha-1 antitrypsin: Implications for osteoarthritis FEBS J (2022) 289, 121-139. PubMed Europe PubMed
Zhang,L.Y., Sun,X.W., Ding,Y.J., Yan,Y.R., Wang,Y., Li,C.X., Li,S.Q., Zhang,L., Song,H.J., Li,H.P. and Li,Q.Y. SERPINA1 Methylation Levels are Associated with Lung Cancer Development in Male Patients with Chronic Obstructive Pulmonary Disease Int J Chron Obstruct Pulmon Dis (2022) 17, 2117-2125. PubMed Europe PubMed DOI PMC EPMC
Zhang,Y., Zhu,M., Zheng,C., Wei,W., Emmer,B.T. and Zhang,B. LMAN1-MCFD2 complex is a cargo receptor for the ER-Golgi transport of alpha1-antitrypsin Biochem J (2022) 479, 839-855. PubMed Europe PubMed DOI PMC EPMC
Zieger,M., Borel,F., Greer,C., Gernoux,G., Blackwood,M., Flotte,T.R. and Mueller,C. Liver-directed SERPINA1 gene therapy attenuates progression of spontaneous and tobacco smoke-induced emphysema in alpha1-antitrypsin null mice Mol Ther Methods Clin Dev (2022) 25, 425-438. PubMed Europe PubMed DOI PMC EPMC

2021

Aiello,M., Ghirardini,M., Marchi,L., Frizzelli,A., Pisi,R., Ferrarotti,I., Bertorelli,G. and Chetta,A. Air Trapping Is Associated with Heterozygosity for Alpha-1 Antitrypsin Mutations in Patients with Asthma Respiration (2021) , 1-10. PubMed Europe PubMed DOI
Ando,Y., Kuroda,A., Kusama,K., Matsutani,T., Matsuda,A. and Tamura,K. Impact of serine protease inhibitor alpha1-antitrypsin on expression of endoplasmic reticulum stress-induced proinflammatory factors in adipocytes Biochem Biophys Rep (2021) 26, 100967-100967. PubMed Europe PubMed DOI PMC EPMC
Azouz,N.P., Klingler,A.M., Callahan,V., Akhrymuk,I.V., Elez,K., Raich,L., Henry,B.M., Benoit,J.L., Benoit,S.W., Noe,F., Kehn-Hall,K. and Rothenberg,M.E. Alpha 1 Antitrypsin is an Inhibitor of the SARS-CoV-2-Priming Protease TMPRSS2 Pathog Immun (2021) 6, 55-74. PubMed Europe PubMed DOI PMC EPMC
Bhakta,V., Hamada,M., Nouanesengsy,A., Lapierre,J., Perruzza,D.L. and Sheffield,W.P. Identification of an alpha-1 antitrypsin variant with enhanced specificity for factor XIa by phage display, bacterial expression, and combinatorial mutagenesis Sci Rep (2021) 11, 5565-5565. PubMed Europe PubMed DOI PMC EPMC
Bhattacharyya,C., Das,C., Ghosh,A., Singh,A.K., Mukherjee,S., Majumder,P.P., Basu,A. and Biswas,N.K. SARS-CoV-2 mutation 614G creates an elastase cleavage site enhancing its spread in high AAT-deficient regions Infect Genet Evol (2021) 90, 104760-104760. PubMed Europe PubMed DOI PMC EPMC
Bianchera,A., Alomari,E. and Bruno,S. Augmentation therapy with alpha 1-antitrypsin: present and future of production, formulation, and delivery Curr Med Chem (2021) PubMed Europe PubMed DOI V
Campbell,R.A., Campbell,H.D., Bircher,J.S., de Araujo,C.V., Denorme,F., Crandell,J.L., Rustad,J.L., Monts,J., Cody,M.J., Kosaka,Y. and Yost,C.C. Placental HTRA1 cleaves alpha-1-antitrypsin to generate a NET-Inhibitory Peptide Blood (2021) PubMed Europe PubMed DOI
Engelmaier,A. and Weber,A. Sensitive and specific measurement of alpha1-antitrypsin activity with an elastase complex formation immunosorbent assay (ECFISA) J Pharm Biomed Anal (2021) , 114476-114476. PubMed Europe PubMed DOI
Fazleen,A. and Wilkinson,T. The emerging role of proteases in alpha1-antitrypsin deficiency and beyond ERJ Open Res (2021) 7 PubMed Europe PubMed DOI PMC EPMC
Ferrarotti,I., Ottaviani,S., Balderacchi,A.M., Barzon,V., De Silvestri,A., Piloni,D., Mariani,F. and Corsico,A.G. COVID-19 infection in severe Alpha 1-antitrypsin deficiency: Looking for a rationale Respir Med (2021) 183, 106440-106440. PubMed Europe PubMed DOI PMC EPMC
Hakim,A., Moll,M., Qiao,D., Liu,J., Lasky-Su,J.A., Silverman,E.K., Vilarinho,S., Jiang,Z.G., Hobbs,B.D. and Cho,M.H. Heterozygosity of the Alpha 1-Antitrypsin Pi*Z Allele and Risk of Liver Disease Hepatol Commun (2021) 5, 1348-1361. PubMed Europe PubMed DOI PMC EPMC
Hamada,M., Bhakta,V., Andres,S.N. and Sheffield,W.P. Stepwise Reversion of Multiply Mutated Recombinant Antitrypsin Reveals a Selective Inhibitor of Coagulation Factor XIa as Active as the M358R Variant Front Cardiovasc Med (2021) 8, 647405-647405. PubMed Europe PubMed DOI PMC EPMC
Hawkins,P., Sya,J., Hup,N.K., Murphy,M.P., McElvaney,N.G. and Reeves,E.P. Alpha-1 Antitrypsin Augmentation Inhibits Proteolysis of Neutrophil Membrane Voltage-Gated Proton Channel-1 in Alpha-1 Deficient Individuals Medicina (Kaunas) (2021) 57 PubMed Europe PubMed DOI PMC EPMC
Hogan,G., Geoghegan,P., Carroll,T.P., Clarke,J., McElvaney,O.F., McElvaney,O.J., McElvaney,N.G. and Curley,G.F. alpha1-Antitrypsin: Key Player or Bystander in Acute Respiratory Distress Syndrome? Anesthesiology (2021) 134, 792-808. PubMed Europe PubMed DOI
Leon,C. and Bouchecareilh,M. The Autophagy Pathway: A Critical Route in the Disposal of Alpha 1-Antitrypsin Aggregates That Holds Many Mysteries Int J Mol Sci (2021) 22 PubMed Europe PubMed DOI PMC EPMC V
Martinez-Gonzalez,C., Blanco,I., Diego,I., Bueno,P. and Miravitlles,M. Estimated Prevalence and Number of PiMZ Genotypes of Alpha-1 Antitrypsin in Seventy-Four Countries Worldwide Int J Chron Obstruct Pulmon Dis (2021) 16, 2617-2630. PubMed Europe PubMed DOI PMC EPMC V
Meza-Segura,M., Birtley,J.R., Maldonado-Contreras,A., Mueller,C., Simin,K.J., Stern,L.J. and McCormick,B.A. SepA Enhances Shigella Invasion of Epithelial Cells by Degrading Alpha-1 Antitrypsin and Producing a Neutrophil Chemoattractant MBio (2021) , e0283321-e0283321. PubMed Europe PubMed DOI PMC EPMC
Ostermann,L., Maus,R., Stolper,J., Schutte,L., Katsarou,K., Tumpara,S., Pich,A., Mueller,C., Janciauskiene,S., Welte,T. and Maus,U.A. Alpha-1 antitrypsin deficiency impairs lung antibacterial immunity in mice JCI Insight (2021) 6 PubMed Europe PubMed DOI
Pini,L., Paoletti,G., Heffler,E., Tantucci,C., Puggioni,F., Corsico,A., Del Giacco,S., Ferri,S., Giordani,J., Paggiaro,P., Pini,A. and Spanevello,A. Alpha1-antitrypsin deficiency and asthma Curr Opin Allergy Clin Immunol (2021) 21, 46-51. PubMed Europe PubMed DOI V
Pini,L., Tiberio,L., Arici,M., Corda,L., Giordani,J., Bargagli,E. and Tantucci,C. Z-alpha1-antitrypsin polymers and small airways disease: a new paradigm in alfa-1 anti-trypsin deficiency-related COPD development? Monaldi Arch Chest Dis (2021) PubMed Europe PubMed DOI
Seixas,S. and Marques,P.I. Known Mutations at the Cause of Alpha-1 Antitrypsin Deficiency an Updated Overview of SERPINA1 Variation Spectrum Appl Clin Genet (2021) 14, 173-194. PubMed Europe PubMed DOI PMC EPMC
Stearns,K., Goldklang,M., Xiao,R., Zelonina,T., Blomenkamp,K., Teckman,J. and D'Armiento,J.M. Knockdown of Alpha-1 Antitrypsin with antisense oligonucleotide does not exacerbate smoke induced lung injury PLoS ONE (2021) 16, e0246040-e0246040. PubMed Europe PubMed DOI PMC EPMC K
Tumpara,S., Korenbaum,E., Kuhnel,M., Jonigk,D., Olejnicka,B., Davids,M., Welte,T., Martinez-Delgado,B. and Janciauskiene,S. A Novel Mouse Monoclonal Antibody C42 against C-Terminal Peptide of Alpha-1-Antitrypsin Int J Mol Sci (2021) 22 PubMed Europe PubMed DOI PMC EPMC
Wettstein,L., Weil,T., Conzelmann,C., Muller,J.A., Gross,R., Hirschenberger,M., Seidel,A., Klute,S., Zech,F., Prelli Bozzo,C., Preising,N., Fois,G., Lochbaum,R., Knaff,P.M., Mailander,V., Standker,L., Thal,D.R., Schumann,C., Stenger,S., Kleger,A., Lochnit,G., Mayer,B., Ruiz-Blanco,Y.B., Hoffmann,M., Sparrer,K.M.J., Pohlmann,S., Sanchez-Garcia,E., Kirchhoff,F., Frick,M. and Munch,J. Alpha-1 antitrypsin inhibits TMPRSS2 protease activity and SARS-CoV-2 infection Nat Commun (2021) 12, 1726-1726. PubMed Europe PubMed DOI PMC EPMC

2020

Attanasio,S., Ferriero,R., Gernoux,G., De Cegli,R., Carissimo,A., Nusco,E., Campione,S., Teckman,J., Mueller,C., Piccolo,P. and Brunetti-Pierri,N. CHOP and c-JUN upregulate the mutant Z alpha-1 antitrypsin, exacerbating its aggregation and liver proteotoxicity J Biol Chem (2020) PubMed Europe PubMed DOI
Aymonnier,K., Kawecki,C., Arocas,V., Boulaftali,Y. and Bouton,M.C. Serpins, New Therapeutic Targets for Hemophilia Thromb Haemost (2020) PubMed Europe PubMed DOI V
Azouz,N.P., Klingler,A.M., Callahan,V., Akhrymuk,I.V., Elez,K., Raich,L., Henry,B.M., Benoit,J.L., Benoit,S.W., Noe,F., Kehn-Hall,K. and Rothenberg,M.E. Alpha 1 Antitrypsin is an Inhibitor of the SARS-CoV-2-Priming Protease TMPRSS2 bioRxiv (2020) PubMed Europe PubMed DOI PMC EPMC
Belchamber,K.B.R., Walker,E.M., Stockley,R.A. and Sapey,E. Monocytes and Macrophages in Alpha-1 Antitrypsin Deficiency Int J Chron Obstruct Pulmon Dis (2020) 15, 3183-3192. PubMed Europe PubMed DOI PMC EPMC V
Bidla,G., Rosenblatt,D.S. and Gilfix,B.M. Identification of Variants in Alpha-1-Antitrypsin by High Resolution Melting J Appl Lab Med (2020) PubMed Europe PubMed DOI
Blanco,I., Diego,I., Bueno,P., Perez-Holanda,S., Casas-Maldonado,F. and Miravitlles,M. Prevalence of alpha1-antitrypsin PiZZ genotypes in patients with COPD in Europe: a systematic review Eur Respir Rev (2020) 29 PubMed Europe PubMed DOI
Bojkova,D., Bechtel,M., McLaughlin,K.M., McGreig,J.E., Klann,K., Bellinghausen,C., Rohde,G., Jonigk,D., Braubach,P., Ciesek,S., Munch,C., Wass,M.N., Michaelis,M. and Cinatl,J., Jr. Aprotinin Inhibits SARS-CoV-2 Replication Cells (2020) 9 PubMed Europe PubMed DOI
Chuang,H.C., Chen,Y.Y., Hsiao,T.C., Chou,H.C., Kuo,H.P., Feng,P.H., Ho,S.C., Chen,J.K., Chuang,K.J. and Lee,K.Y. Alteration in angiotensin-converting enzyme 2 by PM1 during the development of emphysema in rats ERJ Open Res (2020) 6 PubMed Europe PubMed DOI PMC EPMC
Ferrari da Cruz,T., Rufino,R., Lopes,A.J., Noronha,A., Medeiros Anselmo,F. and Henrique da Costa,C. Evaluation of the Small Airways in Patients with Chronic Obstructive Pulmonary Disease and Alpha-1 Antitrypsin Deficiency Int J Chron Obstruct Pulmon Dis (2020) 15, 2267-2274. PubMed Europe PubMed DOI PMC EPMC
Jagger,A.M., Waudby,C.A., Irving,J.A., Christodoulou,J. and Lomas,D.A. High-resolution ex vivo NMR spectroscopy of human Z alpha1-antitrypsin Nat Commun (2020) 11, 6371-6371. PubMed Europe PubMed DOI PMC EPMC
Lechowicz,U., Rudzinski,S., Jezela-Stanek,A., Janciauskiene,S. and Chorostowska-Wynimko,J. Post-Translational Modifications of Circulating Alpha-1-Antitrypsin Protein Int J Mol Sci (2020) 21 PubMed Europe PubMed DOI PMC EPMC V
Lior,Y., Jasevitch,M., Ochayon,D.E., Zaretsky,M., Lewis,E.C. and Aharoni,A. Application of directed evolution and back-to-consensus algorithms to human alpha1-antitrypsin leads to diminished anti-protease activity and augmented anti-inflammatory activities Cell Immunol (2020) 355, 104135-104135. PubMed Europe PubMed DOI
Lopez-Campos,J.L., Carrasco Hernandez,L. and Caballero Eraso,C. Implications of a Change of Paradigm in Alpha1 Antitrypsin Deficiency Augmentation Therapy: From Biochemical to Clinical Efficacy J Clin Med (2020) 9 PubMed Europe PubMed DOI PMC EPMC V
McNulty,M.J., Silberstein,D.Z., Kuhn,B.T., Padgett,H.S., Nandi,S., McDonald,K.A. and Cross,C.E. Alpha-1 antitrypsin deficiency and recombinant protein sources with focus on plant sources: Updates, challenges and perspectives Free Radic Biol Med (2020) 163, 10-30. PubMed Europe PubMed DOI V
Park,J.E., Tran,T.X.T., Park,N., Yeom,J., Kim,K. and Kang,M.J. The Function of Drosophila USP14 in Endoplasmic Reticulum Stress and Retinal Degeneration in a Model for Autosomal Dominant Retinitis Pigmentosa Biology (Basel) (2020) 9 PubMed Europe PubMed DOI
Perez-Rubio,G., Ambrocio-Ortiz,E., Lopez-Flores,L.A., Juarez-Martin,A.I., Jimenez-Valverde,L.O., Zoreque-Cabrera,S., Galicia-Negrete,G., Ramirez-Diaz,M.E., Cruz-Vicente,F., Castillejos-Lopez,M.J., Salinas-Lara,C., Hernandez-Zenteno,R.J., Ramirez-Venegas,A. and Falfan-Valencia,R. Heterozygous Genotype rs17580 AT (PiS) in SERPINA1 is Associated with COPD Secondary to Biomass-Burning and Tobacco Smoking: A Case-Control and Populational Study Int J Chron Obstruct Pulmon Dis (2020) 15, 1181-1190. PubMed Europe PubMed DOI PMC EPMC
Rottier,S.J., Dreuning,L.C., van Pelt,J., van Geloven,A.A.W., Beele,X.D.Y., Huisman,P.M., Deurholt,W.Y., Rottier,C.A., van Leeuwen,K., de Boer,M., van Mierlo,G., Boermeester,M.A.A. and Schreurs,W.H. Alpha-1-Antitrypsin deficiency (carrier) as possible risk factor for development of colonic diverticula. A multicentre prospective case-control study: The ALADDIN study Colorectal Dis (2020) PubMed Europe PubMed DOI
Scott,B.M. and Sheffield,W.P. Engineering the serpin alpha1-antitrypsin: a diversity of goals and techniques Protein Sci (2020) 29, 856-871. PubMed Europe PubMed DOI
Scott,B.M., Matochko,W.L., Gierczak,R.F., Bhakta,V., Derda,R. and Sheffield,W.P. 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 PLoS ONE (2020) 15, e0238969-e0238969. PubMed Europe PubMed DOI PMC EPMC
Shapira,G., Shomron,N. and Gurwitz,D. Ethnic differences in alpha-1 antitrypsin deficiency allele frequencies may partially explain national differences in COVID-19 fatality rates FASEB J (2020) 34, 14160-14165. PubMed Europe PubMed DOI PMC EPMC
Sosulski,M.L., Stiles,K.M., Frenk,E.Z., Hart,F.M., Matsumura,Y., De,B.P., Kaminsky,S.M. and Crystal,R.G. Gene therapy for alpha 1-antitrypsin deficiency with an oxidant-resistant human alpha 1-antitrypsin JCI Insight (2020) 5 PubMed Europe PubMed DOI
Stockley,R.A. Alpha-1 antitrypsin deficiency: have we got the right proteinase? Chronic Obstr Pulm Dis (2020) 7 PubMed Europe PubMed DOI
Tumpara,S., Martinez-Delgado,B., Gomez-Mariano,G., Liu,B., DeLuca,D.S., Korenbaum,E., Jonigk,D., Jugert,F., Wurm,F.M., Wurm,M.J., Welte,T. and Janciauskiene,S. The Delivery of alpha1-Antitrypsin Therapy Through Transepidermal Route: Worthwhile to Explore Front Pharmacol (2020) 11, 983-983. PubMed Europe PubMed DOI PMC EPMC
Yang,C., Keshavjee,S. and Liu,M. Alpha-1 Antitrypsin for COVID-19 Treatment: Dual Role in Antiviral Infection and Anti-Inflammation Front Pharmacol (2020) 11, 615398-615398. PubMed Europe PubMed DOI PMC EPMC

2019

Campos,M.A., Geraghty,P., Holt,G., Mendes,E., Newby,P.R., Ma,S., Luna-Diaz,L.V., Turino,G.M. and Stockley,R.A. The biological effects of double-dose alpha-1 antitrypsin augmentation therapy. A pilot clinical trial Am J Respir Crit Care Med (2019) 200, 318-326. PubMed Europe PubMed DOI U
Zhang,N., Wright,T., Caraway,P. and Xu,J. Enhanced secretion of human alpha1-antitrypsin expressed with a novel glycosylation module in tobacco BY-2 cell culture Bioengineered (2019) 10, 87-97. PubMed Europe PubMed DOI E

2018

Chapman,K.R., Chorostowska-Wynimko,J., Koczulla,A.R., Ferrarotti,I. and McElvaney,N.G. Alpha 1 antitrypsin to treat lung disease in alpha 1 antitrypsin deficiency: recent developments and clinical implications Int J Chron Obstruct Pulmon Dis (2018) 13, 419-432. PubMed Europe PubMed DOI PMC EPMC U
Giri Rao,V.V.H. and Gosavi,S. On the folding of a structurally complex protein to its metastable active state Proc Natl Acad Sci U S A (2018) 115, 1998-2003. PubMed Europe PubMed DOI S
Julicher,K., Wahner,A., Haase,K., Barbour,K.W., Berger,F.G., Wiehlmann,L., Davenport,C., Schuster-Gossler,K., Stitz,J., Cantz,T. and Eggenschwiler,R. Functional characterization of the mouse Serpina1 paralog DOM-7 Biol Chem (2018) 399, 577-582. PubMed Europe PubMed DOI
Lior,Y., Zaretsky,M., Ochayon,D.E., Lotysh,D., Baranovski,B.M., Schuster,R., Guttman,O., Aharoni,A. and Lewis,E.C. Point mutation of a non-elastase-binding site in human alpha1-antitrypsin alters its anti-inflammatory properties Front Immunol (2018) 9, 759-759. PubMed Europe PubMed DOI PMC EPMC
Morifuji,Y., Xu,J., Karasaki,N., Iiyama,K., Morokuma,D., Hino,M., Masuda,A., Yano,T., Mon,H., Kusakabe,T. and Lee,J.M. Expression, purification, and characterization of recombinant human alpha1-antitrypsin produced using silkworm-baculovirus expression system Mol Biotechnol (2018) 60, 924-934. PubMed Europe PubMed DOI E

2017

Andersen,O.J., Risor,M.W., Poulsen,E.C., Nielsen,N.C., Miao,Y., Enghild,J.J. and Schiott,B. Reactive center loop insertion in alpha-1-antitrypsin captured by accelerated molecular dynamics simulation Biochemistry (2017) 56, 634-646. PubMed Europe PubMed DOI S
Boerema,D.J., An,B., Gandhi,R.P., Papineau,R., Regnier,E., Wilder,A., Molitor,A., Tang,A.P. and Kee,S.M. Biochemical comparison of four commercially available human alpha1-proteinase inhibitors for treatment of alpha1-antitrypsin deficiency Biologicals (2017) 50, 63-72. PubMed Europe PubMed DOI U
Carpagnano,G.E., Santacroce,R., Palmiotti,G.A., Leccese,A., Giuffreda,E., Margaglione,M., Foschino Barbaro,M.P., Aliberti,S. and Lacedonia,D. A new SERPINA-1 missense mutation associated with alpha-1 antitrypsin deficiency and bronchiectasis Lung (2017) 195, 679-682. PubMed Europe PubMed DOI M
Chung,H.S., Kim,J.S., Lee,S.M. and Park,S.J. Role of the P2 residue of human alpha 1-antitrypsin in determining target protease specificity PLoS ONE (2017) 12, e0185074-e0185074. PubMed Europe PubMed DOI PMC EPMC
Crystal,R.G. Compelling evidence for the efficacy of alpha1-antitrypsin augmentation treatment for alpha1-antitrypsin deficiency Lancet Respir Med (2017) 5, 7-8. PubMed Europe PubMed DOI U
Huangfu,C., Zhang,J., Ma,Y., Jia,J., Lv,M., Zhao,X. and Zhang,J. New process for purifying high purity alpha1-antitrypsin from Cohn Fraction IV by chromatography: a promising method for the better utilization of plasma J Chromatogr B Analyt Technol Biomed Life Sci (2017) 1046, 156-164. PubMed Europe PubMed DOI U
Kee,S., Weber,D., Popp,B., Nowak,T., Schafer,W., Groner,A. and Roth,N.J. Pathogen safety and characterisation of a highly purified human alpha1-proteinase inhibitor preparation Biologicals (2017) 47, 25-32. PubMed Europe PubMed DOI U
Li,W., Wang,Q., Li,S., Jiang,A. and Sun,W. Molecular cloning, genomic structure, polymorphism analysis and recombinant expression of a alpha1-antitrypsin like gene from swamp eel, Monopterus albus Fish Shellfish Immunol (2017) 62, 124-138. PubMed Europe PubMed DOI
McElvaney,N.G., Burdon,J., Holmes,M., Glanville,A., Wark,P.A., Thompson,P.J., Hernandez,P., Chlumsky,J., Teschler,H., Ficker,J.H., Seersholm,N., Altraja,A., Makitaro,R., Chorostowska-Wynimko,J., Sanak,M., Stoicescu,P.I., Piitulainen,E., Vit,O., Wencker,M., Tortorici,M.A., Fries,M., Edelman,J.M. and Chapman,K.R. Long-term efficacy and safety of alpha1 proteinase inhibitor treatment for emphysema caused by severe alpha1 antitrypsin deficiency: an open-label extension trial (RAPID-OLE) Lancet Respir Med (2017) 5, 51-60. PubMed Europe PubMed DOI U
Parr,D.G. and Lara,B. Clinical utility of alpha-1 proteinase inhibitor in the management of adult patients with severe alpha-1 antitrypsin deficiency: a review of the current literature Drug Des Devel Ther (2017) 11, 2149-2162. PubMed Europe PubMed DOI PMC EPMC U

2016

Akbas,N., Gonzalez,G., Buffone,G.J., Grenache,D.G. and Devaraj,S. A library of rare alpha1-antitrypsin (AAT) variant phenotypes to aid in the diagnosis of AAT deficiency Am J Clin Pathol (2016) 146, 289-293. PubMed Europe PubMed DOI M
Gogol,M., Ostrowska,D., Klaga,K., Bochenska,O., Wolak,N., Aoki,W., Ueda,M., Kozik,A. and Rapala-Kozik,M. Inactivation of alpha1-proteinase inhibitor by Candida albicans aspartic proteases favors the epithelial and endothelial cell colonization in the presence of neutrophil extracellular traps Acta Biochim Pol (2016) 63, 167-175. PubMed Europe PubMed DOI
Huang,X., Zheng,Y., Zhang,F., Wei,Z., Wang,Y., Carrell,R.W., Read,R.J., Chen,G.-Q. and Zhou,A. Molecular mechanism of Z alpha1-antitrypsin deficiency J Biol Chem (2016) 291, 15674-15686. PubMed Europe PubMed DOI M
Jha,S., Agarwal,S., Sanyal,I. and Amla,D.V. Single-step purification and characterization of a recombinant serine proteinase inhibitor from transgenic plants Appl Biochem Biotechnol (2016) 179, 220-236. PubMed Europe PubMed DOI E U
Lomas,D.A., Hurst,J.R. and Gooptu,B. Update on alpha-1 antitrypsin deficiency: new therapies J Hepatol (2016) 65, 413-424. PubMed Europe PubMed DOI M
Motamedi-Shad,N., Jagger,A.M., Liedtke,M., Faull,S.V., Nanda,A.S., Salvadori,E., Wort,J.L., Kay,C.W.M., Heyer-Chauhan,N., Miranda,E., Perez,J., Ordonez,A., Haq,I., Irving,J.A. and Lomas,D.A. An antibody that prevents serpin polymerisation acts by inducing a novel allosteric behavior Biochem J (2016) 473, 3269-3290. PubMed Europe PubMed DOI
Perlmutter,D.H. alpha1-Antitrypsin deficiency: a misfolded secretory protein variant with unique effects on the endoplasmic reticulum Endoplasmic Reticulum Stress Dis (2016) 3, 63-72. PubMed Europe PubMed DOI PMC EPMC M
Sheffield,W.P. and Bhakta,V. The M358R variant of alpha(1)-proteinase inhibitor inhibits coagulation factor VIIa Biochem Biophys Res Commun (2016) 470, 710-713. PubMed Europe PubMed DOI M
Zhou,X. and Declerck,P.J. Prevention of serpin misfolding by RNA aptamers Cell Chem Biol (2016) 23, 639-640. PubMed Europe PubMed DOI I

2015

Berthelier,V., Harris,J.B., Estenson,K.N. and Baudry,J. Discovery of an inhibitor of Z-alpha1 antitrypsin polymerization PLoS ONE (2015) 10, 0126256- PubMed Europe PubMed DOI
Gierczak,R.F., Bhakta,V., Xie,M. and Sheffield,W.P. Comparison of mammalian and bacterial expression library screening to detect recombinant alpha-1 proteinase inhibitor variants with enhanced thrombin inhibitory capacity J Biotechnol (2015) 208, 54-62. PubMed Europe PubMed DOI M
Parambeth,J.C., Suchodolski,J.S. and Steiner,J.M. Purification and partial characterization of alpha1-proteinase inhibitor in the common marmoset (Callithrix jacchus) Res Vet Sci (2015) 99, 17-22. PubMed Europe PubMed DOI
Rahman,M.A., Mitra,S., Sarkar,A. and Wewers,M.D. Alpha 1-antitrypsin does not inhibit human monocyte caspase-1 PLoS ONE (2015) 10, e0117330-e0117330. PubMed Europe PubMed DOI PMC EPMC
Shi,C., Zhang,G., Han,B., Yang,J., Liu,H. and Xi,J. [Effects of alpha1-PDX, a furin inhibitor, on growth, invasion, and tumorigenicity of cervical cancer HeLa cells] Nan Fang Yi Ke Da Xue Xue Bao (2015) 35, 432-436. PubMed Europe PubMed

2014

Behrens,M.A., Sendall,T.J., Pedersen,J.S., Kjeldgaard,M., Huntington,J.A. and Jensen,J.K. The shapes of Z-alpha1-antitrypsin polymers in solution support the C-terminal domain-swap mechanism of polymerization Biophys J (2014) 107, 1905-1912. PubMed Europe PubMed DOI M S
Chiuchiolo,M.J., Kaminsky,S.M., Sondhi,D., Mancenido,D., Hollmann,C. and Crystal,R.G. Phase I/II study of intrapleural administration of a serotype rh.10 replication-deficient adeno-associated virus gene transfer vector expressing the human alpha1-antitrypsin cDNA to individuals with alpha1-antitrypsin deficiency Hum Gene Ther Clin Develop (2014) 25, 112-133. PubMed Europe PubMed DOI U
Ferrarotti,I., Carroll,T.P., Ottaviani,S., Fra,A.M., O Brien,G., Molloy,K., Corda,L., Medicina,D., Curran,D.R., McElvaney,N.G. and Luisetti,M. Identification and characterisation of eight novel SERPINA1 null mutations Orphanet J Rare Dis (2014) 9, 172-172. PubMed Europe PubMed DOI PMC EPMC M
Frochaux,V., Hildebrand,D., Talke,A., Linscheid,M.W. and Schluter,H. Alpha-1-antitrypsin: a novel human high temperature requirement protease A1 (HTRA1) substrate in human placental tissue PLoS ONE (2014) 9, e109483-e109483. PubMed Europe PubMed DOI PMC EPMC
Ghouse,R., Chu,A., Wang,Y. and Perlmutter,D.H. Mysteries of alpha1-antitrypsin deficiency: emerging therapeutic strategies for a challenging disease Dis Model Mech (2014) 7, 411-419. PubMed Europe PubMed DOI PMC EPMC M V
Gierczak,R.F., Pepler,L., Bhagirath,V., Liaw,P.C. and Sheffield,W.P. Alpha-1 proteinase inhibitor M358R reduces thrombin generation when displayed on the surface of cells expressing tissue factor Thromb Res (2014) 134, 1142-1149. PubMed Europe PubMed DOI U
Gooptu,B., Dickens,J.A. and Lomas,D.A. The molecular and cellular pathology of alpha1-antitrypsin deficiency Trends Mol Med (2014) 20, 116-127. PubMed Europe PubMed DOI M
Jha,S., Sanyal,I. and Amla,D.V. Single amino acid substitutions in recombinant plant-derived human alpha1-proteinase inhibitor confer enhanced stability and functional efficacy Biochim Biophys Acta (2014) 1840, 416-427. PubMed Europe PubMed DOI E U
Joly,P., Lacan,P., Chapuis-Cellier,C., Garcia,C., Bererd,M. and Francina,A. Molecular characterization of 7 new alpha-1 anti-trypsin (A1AT) variants including two with an associated deficient phenotype Clin Chim Acta (2014) 427, 21-22. PubMed Europe PubMed DOI M
Lara,B., Martinez,M.T., Blanco,I., Hernandez-Moro,C., Velasco,E.A., Ferrarotti,I., Rodriguez-Frias,F., Perez,L., Vazquez,I., Alonso,J., Posada,M. and Martinez-Delgado,B. Severe alpha-1 antitrypsin deficiency in composite heterozygotes inheriting a new splicing mutation QOMadrid Respir Res (2014) 15, 125-125. PubMed Europe PubMed DOI M
Liu,L., Werner,M. and Gershenson,A. Collapse of a long axis: single-molecule Forster resonance energy transfer and serpin equilibrium unfolding Biochemistry (2014) 53, 2903-2914. PubMed Europe PubMed DOI
Ljujic,M., Divac Rankov,A., Kojic,S., Miranda,E. and Radojkovic,D. Functional analysis of novel alpha-1 antitrypsin variants G320R and V321F Mol Biol Rep (2014) 41, 6133-6141. PubMed Europe PubMed DOI M
Sinden,N.J., Koura,F. and Stockley,R.A. The significance of the F variant of alpha-1-antitrypsin and unique case report of a PiFF homozygote BMC Pulm Med (2014) 14, 132-132. PubMed Europe PubMed DOI M
Yang,L., Dinarvand,P., Qureshi,S.H. and Rezaie,A.R. Engineering D-helix of antithrombin in alpha-1-proteinase inhibitor confers antiinflammatory properties on the chimeric serpin Thromb Haemost (2014) 112, 164-175. PubMed Europe PubMed DOI E

2013

Hada,K., Isshiki,K., Matsuda,S., Yuasa,K. and Tsuji,A. Engineering of alpha1-antitrypsin variants with improved specificity for the proprotein convertase furin using site-directed random mutagenesis Protein Eng Des Sel (2013) 26, 123-131. PubMed Europe PubMed DOI
Haq,I., Irving,J.A., Faull,S.V., Dickens,J.A., Ordnez,A., Belorgey,D., Gooptu,B. and Lomas,D.A. Reactive centre loop mutants of alpha-1-antitrypsin reveal position-specific effects on intermediate formation along the polymerization pathway Biosci Rep (2013) 33, e00046- PubMed Europe PubMed DOI
Izaguirre,G., Qi,L., Lima,M. and Olson,S.T. Identification of serpin determinants of specificity and selectivity for furin inhibition through studies of alpha1PDX (alpha1-protease inhibitor Portland)-serpin B8 and furin active-site loop chimeras J Biol Chem (2013) 288, 21802-21814. PubMed Europe PubMed DOI
Laurell,C.B. and Eriksson,S. The electrophoretic alpha1-globulin pattern of serum in alpha1-antitrypsin deficiency. 1963 COPD (2013) 10 Suppl 1, 3-8. PubMed Europe PubMed DOI M
Lee,K.J., Lee,S.M., Gil,J.Y., Kwon,O., Kim,J.Y., Park,S.J., Chung,H.S. and Oh,D.B. N-glycan analysis of human alpha1-antitrypsin produced in Chinese hamster ovary cells Glycoconj J (2013) 30, 537-547. PubMed Europe PubMed DOI E
Maddur,A.A., Swanson,R., Izaguirre,G., Gettins,P.G. and Olson,S.T. Kinetic intermediates en route to the final serpin-protease complex: studies of complexes of alpha1-protease inhibitor with trypsin J Biol Chem (2013) 288, 32020-32035. PubMed Europe PubMed DOI
Mansuy-Aubert,V., Zhou,Q.L., Xie,X., Gong,Z., Huang,J.Y., Khan,A.R., Aubert,G., Candelaria,K., Thomas,S., Shin,D.J., Booth,S., Baig,S.M., Bilal,A., Hwang,D., Zhang,H., Lovell-Badge,R., Smith,S.R., Awan,F.R. and Jiang,Z.Y. Imbalance between neutrophil elastase and its inhibitor alpha1-antitrypsin in obesity alters insulin sensitivity, inflammation, and energy expenditure Cell Metab (2013) 17, 534-548. PubMed Europe PubMed DOI I
Mueller,C. and Flotte,T.R. Gene-based therapy for alpha-1 antitrypsin deficiency COPD (2013) 10 Suppl 1, 44-49. PubMed Europe PubMed DOI U
Sandhaus,R.A. and Stoller,J.K. Introduction to the 50th anniversary of the description of alpha-1 antitrypsin deficiency COPD (2013) 10 Suppl 1, 1-2. PubMed Europe PubMed DOI M
Stockley,R.A., Dirksen,A. and Stolk,J. Alpha-1 antitrypsin deficiency: The European experience COPD (2013) 10 Suppl 1, 50-53. PubMed Europe PubMed DOI M
Stoller,J.K. and Brantly,M. The challenge of detecting alpha-1 antitrypsin deficiency COPD (2013) 10 Suppl 1, 26-34. PubMed Europe PubMed DOI M
Wang,Z., Hilder,T.L., van der Drift,K., Sloan,J. and Wee,K. Structural characterization of recombinant alpha-1-antitrypsin expressed in a human cell line Anal Biochem (2013) 437, 20-28. PubMed Europe PubMed DOI E
Wewers,M.D. and Crystal,R.G. Alpha-1 antitrypsin augmentation therapy COPD (2013) 10 Suppl 1, 64-67. PubMed Europe PubMed DOI M

2012

Dolmer,K. and Gettins,P.G. How the serpin alpha1-proteinase inhibitor folds J Biol Chem (2012) 287, 12425-12432. PubMed Europe PubMed DOI
Enewold,L., Mechanic,L.E., Bowman,E.D., Platz,E.A. and Alberg,A.J. SERPINA1 and ELA2 Polymorphisms Are Not Associated with COPD or Lung Cancer Anticancer Res (2012) 32, 3923-3928. PubMed Europe PubMed M
Kass,I., Knaupp,A.S., Bottomley,S.P. and Buckle,A.M. Conformational properties of the disease-causing Z variant of alpha1-antitrypsin revealed by theory and experiment Biophys J (2012) 102, 2856-2865. PubMed Europe PubMed DOI PMC EPMC M
McNab,G.L., Dafforn,T.R., Wood,A., Sapey,E. and Stockley,R.A. A novel model and molecular therapy for Z alpha-1 antitrypsin deficiency Mamm Genome (2012) 23, 241-249. PubMed Europe PubMed DOI M
Miravitlles,M. Alpha-1-antitrypsin and other proteinase inhibitors Curr Opin Pharmacol (2012) 12, 309-314. PubMed Europe PubMed DOI U
Nyon,M.P., Kirkpatrick,J., Cabrita,L.D., Christodoulou,J. and Gooptu,B. 1H, 15N and 13C backbone resonance assignments of the archetypal serpin alpha1-antitrypsin Biomol NMR Assign (2012) 6, 153-156. PubMed Europe PubMed DOI S
Qamar,A.A., Burke,S.K., Lafleur,J.D., Ding,B.C., Bland,K.S., Wong,M.D., Gustafson,P.N., Blair,A.T. and Franano,F.N. The ability of serum from alpha 1-antitrypsin-deficient patients to inhibit PRT-201, a recombinant human type I pancreatic elastase Biotechnol Appl Biochem (2012) 59, 22-28. PubMed Europe PubMed DOI
Ross,D., Brown,T., Harper,R., Pamarthi,M., Nixon,J., Bromirski,J., Li,C.M., Ghali,R., Xie,H., Medvedeff,G., Li,H., Scuderi,P., Arora,V., Hunt,J. and Barnett,T. Production and characterization of a novel human recombinant alpha-1-antitrypsin in PER.C6 cells J Biotechnol (2012) 162, 262-273. PubMed Europe PubMed DOI E
Saunders,D.N., Tindall,E.A., Shearer,R.F., Roberson,J., Decker,A., Wilson,J.A. and Hayes,V.M. A novel SERPINA1 mutation causing serum alpha(1)-antitrypsin deficiency PLoS ONE (2012) 7, e51762-e51762. PubMed Europe PubMed DOI PMC EPMC M
Sclar,D.A., Evans,M.A., Robison,L.M. and Skaer,T.L. alpha1-Proteinase inhibitor (human) in the treatment of hereditary emphysema secondary to alpha1-antitrypsin deficiency: number and costs of years of life gained Clin Drug Investig (2012) 32, 353-360. PubMed Europe PubMed DOI U
Stocks,B.B., Sarkar,A., Wintrode,P.L. and Konermann,L. Early hydrophobic collapse of alpha1-antitrypsin facilitates formation of a metastable state: insights from oxidative labeling and mass spectrometry J Mol Biol (2012) 423, 789-799. PubMed Europe PubMed DOI
Tsutsui,Y., Dela Cruz,R. and Wintrode,P.L. Folding mechanism of the metastable serpin alpha1-antitrypsin Proc Natl Acad Sci U S A (2012) 109, 4467-4472. PubMed Europe PubMed DOI
Wang,Y., He,Y., Abraham,B., Rouhani,F.N., Brantly,M.L., Scott,D.E. and Reed,J.L. Cytosolic, autocrine alpha-1 proteinase inhibitor (A1PI) inhibits caspase-1 and blocks IL-1beta dependent cytokine release in monocytes PLoS ONE (2012) 7, e51078-e51078. PubMed Europe PubMed DOI PMC EPMC
Zhang,L., Shi,J., Jiang,D., Stupak,J., Ou,J., Qiu,Q., An,N., Li,J. and Yang,D. Expression and characterization of recombinant human alpha-antitrypsin in transgenic rice seed J Biotechnol (2012) 164, 300-308. PubMed Europe PubMed DOI E

2011

Dickens,J.A. and Lomas,D.A. Why has it been so difficult to prove the efficacy of alpha-1-antitrypsin replacement therapy? Insights from the study of disease pathogenesis Drug Des Devel Ther (2011) 5, 391-405. PubMed Europe PubMed DOI PMC EPMC U
Flotte,T.R. and Mueller,C. Gene therapy for alpha-1 antitrypsin deficiency Hum Mol Genet (2011) 20, R87-R92. PubMed Europe PubMed DOI U
Gierczak,R.F., Sutherland,J.S., Bhakta,V., Toltl,L.J., Liaw,P.C. and Sheffield,W.P. Retention of thrombin inhibitory activity by recombinant serpins expressed as integral membrane proteins tethered to the surface of mammalian cells J Thromb Haemost (2011) 9, 2424-2435. PubMed Europe PubMed DOI
Huntington,J.A. Serpin structure, function and dysfunction J Thromb Haemost (2011) 9 Suppl 1, 26-34. PubMed Europe PubMed DOI S
Janciauskiene,S.M., Bals,R., Koczulla,R., Vogelmeier,C., Kohnlein,T. and Welte,T. The discovery of alpha1-antitrypsin and its role in health and disease Respir Med (2011) 105, 1129-1139. PubMed Europe PubMed DOI V
Knaupp,A.S. and Bottomley,S.P. Structural change in beta-sheet A of Z alpha(1)-antitrypsin is responsible for accelerated polymerization and disease J Mol Biol (2011) 413, 888-898. PubMed Europe PubMed DOI M
Krishnan,B. and Gierasch,L.M. Dynamic local unfolding in the serpin alpha-1 antitrypsin provides a mechanism for loop insertion and polymerization Nat Struct Mol Biol (2011) 18, 222-226. PubMed Europe PubMed DOI
Maciejewska-Radomska,A. and Wilkowska,A. Panniculitis as a rare clinical manifestation of genetically determined abnormalities of alpha I-antitrypsin - review of the literature Prz Dermatol (2011) 98, 350-354. M V
Patschull,A.O., Segu,L., Nyon,M.P., Lomas,D.A., Nobeli,I., Barrett,T.E. and Gooptu,B. Therapeutic target-site variability in alpha1-antitrypsin characterized at high resolution Acta Crystallogr Sect F Struct Biol Cryst Commun (2011) 67, 1492-1497. PubMed Europe PubMed DOI PMC EPMC S
Perez,P., Adriaansen,J., Goldsmith,C.M., Zheng,C. and Baum,B.J. Transgenic alpha-1-antitrypsin secreted into the bloodstream from salivary glands is biologically active Oral Dis (2011) 17, 476-483. PubMed Europe PubMed DOI U
Sarkar,A. and Wintrode,P.L. Effects of glycosylation on the stability and flexibility of a metastable protein: the human serpin alpha1-antitrypsin Int J Mass Spectrom (2011) 302, 69-75. PubMed Europe PubMed DOI
Thelwell,C., Marszal,E., Rigsby,P. and Longstaff,C. An international collaborative study to establish the WHO 1st international standard for alpha-1-antitrypsin Vox Sang (2011) 101, 83-89. PubMed Europe PubMed DOI U
Vukoti,K.M., Kadiyala,C.S. and Miyagi,M. Streptomyces erythraeus trypsin inactivates alpha1-antitrypsin FEBS Lett (2011) 585, 3898-3902. PubMed Europe PubMed DOI
Wang,D., Wang,W., Dawkins,P., Paterson,T., Kalsheker,N., Sallenave,J.M. and McGarry Houghton,A. Deletion of serpina1a, a murine alpha1-antitrypsin ortholog, results in embryonic lethality Exp Lung Res (2011) 37, 291-300. PubMed Europe PubMed DOI K
Yamasaki,M., Sendall,T.J., Pearce,M.C., Whisstock,J.C. and Huntington,J.A. Molecular basis of alpha1-antitrypsin deficiency revealed by the structure of a domain-swapped trimer EMBO Rep (2011) 12, 1011-1017. PubMed Europe PubMed DOI M

2010

Agarwal,S., Jha,S., Sanyal,I. and Amla,D.V. Expression and purification of recombinant human alpha1-proteinase inhibitor and its single amino acid substituted variants in Escherichia coli for enhanced stability and biological activity J Biotechnol (2010) 147, 64-72. PubMed Europe PubMed DOI E
Huntington,J.A. and Whisstock,J.C. Molecular contortionism - on the physical limits of serpin 'loop-sheet' polymers Biol Chem (2010) 391, 973-982. PubMed Europe PubMed DOI
Kilty,S.J., Bosse,Y., Cormier,C., Endam,L.M. and Desrosiers,M.Y. Polymorphisms in the SERPINA1 (alpha-1-antitrypsin) gene are associated with severe chronic rhinosinusitis unresponsive to medical therapy Am J Rhinol Allergy (2010) 24, e4-e9. PubMed Europe PubMed DOI M
Knaupp,A.S., Levina,V., Robertson,A.L., Pearce,M.C. and Bottomley,S.P. Kinetic instability of the serpin Z alpha1-antitrypsin promotes aggregation J Mol Biol (2010) 396, 375-383. PubMed Europe PubMed DOI M
Ratner,M. Cochrane meta-analysis on alpha-1 antitrypsin prompts furor Nat Biotechnol (2010) 28, 885-886. PubMed Europe PubMed DOI U
Yamasaki,M., Sendall,T.J., Harris,L.E., Lewis,G.M. and Huntington,J.A. Loop-sheet mechanism of serpin polymerization tested by reactive center loop mutations J Biol Chem (2010) 285, 30752-30758. PubMed Europe PubMed DOI

2009

Baek,J.H., Yang,W.S., Lee,C. and Yu,M.H. Functional unfolding of alpha1-antitrypsin probed by hydrogen-deuterium exchange coupled with mass spectrometry Mol Cell Proteomics (2009) 8, 1072-1081. PubMed Europe PubMed DOI PMC EPMC
Chill,L., Trinh,L., Azadi,P., Ishihara,M., Sonon,R., Karnaukhova,E., Ophir,Y., Golding,B. and Shiloach,J. Production, purification, and characterization of human alpha1 proteinase inhibitor from Aspergillus niger Biotechnol Bioeng (2009) 102, 828-844. PubMed Europe PubMed DOI E
Gooptu,B., Miranda,E., Nobeli,I., Mallya,M., Purkiss,A., Brown,S.C., Summers,C., Phillips,R.L., Lomas,D.A. and Barrett,T.E. Crystallographic and cellular characterisation of two mechanisms stabilising the native fold of alpha1-antitrypsin: implications for disease and drug design J Mol Biol (2009) 387, 857-868. PubMed Europe PubMed DOI PMC EPMC S
Levina,V., Dai,W., Knaupp,A.S., Kaiserman,D., Pearce,M.C., Cabrita,L.D., Bird,P.I. and Bottomley,S.P. Expression, purification and characterization of recombinant Z alpha(1)-antitrypsin - the most common cause of alpha(1)-antitrypsin deficiency Protein Expr Purif (2009) 68, 226-232. PubMed Europe PubMed DOI E M
Maisa,A., Stroher,U., Klenk,H.D., Garten,W. and Strecker,T. Inhibition of Lassa virus glycoprotein cleavage and multicycle replication by site 1 protease-adapted alpha(1)-antitrypsin variants PLoS Negl Trop Dis (2009) 3, e446-e446. PubMed Europe PubMed DOI PMC EPMC
Mickowska,B. Purification and characterization of alpha(1)-proteinase inhibitor and antithrombin III: major serpins of rainbow trout (Oncorhynchuss mykiss) and carp (Cyprinus carpio) blood plasma Fish Physiol Biochem (2009) 35, 231-240. PubMed Europe PubMed DOI
Nadai,M., Bally,J., Vitel,M., Job,C., Tissot,G., Botterman,J. and Dubald,M. High-level expression of active human alpha1-antitrypsin in transgenic tobacco chloroplasts Transgenic Res (2009) 18, 173-183. PubMed Europe PubMed DOI E
Sengupta,T., Tsutsui,Y. and Wintrode,P.L. Local and global effects of a cavity filling mutation in a metastable serpin Biochemistry (2009) 48, 8233-8240. PubMed Europe PubMed DOI PMC EPMC

2008

Fregonese,L., Stolk,J., Frants,R.R. and Veldhuisen,B. Alpha-1 antitrypsin Null mutations and severity of emphysema Respir Med (2008) 102, 876-884. PubMed Europe PubMed DOI M
Matthiessen,H.P., Willemse,J., Weber,A., Turecek,P.L., Deiteren,K., Hendriks,D., Ehrlich,H.J. and Schwarz,H.P. Ethanol dependence of alpha1-antitrypsin C-terminal Lys truncation mediated by basic carboxypeptidases Transfusion (2008) 48, 314-320. PubMed Europe PubMed DOI
Pearce,M.C., Morton,C.J., Feil,S.C., Hansen,G., Adams,J.J., Parker,M.W. and Bottomley,S.P. Preventing serpin aggregation: the molecular mechanism of citrate action upon antitrypsin unfolding Protein Sci (2008) 17, 2127-2133. PubMed Europe PubMed DOI PMC EPMC S
Prins,J., van der Meijden,B.B., Kraaijenhagen,R.J. and Wielders,J.P. Inherited chronic obstructive pulmonary disease: new selective-sequencing workup for alpha1-antitrypsin deficiency identifies 2 previously unidentified null alleles Clin Chem (2008) 54, 101-107. PubMed Europe PubMed DOI M
Sandhaus,R.A., Turino,G., Stocks,J., Strange,C., Trapnell,B.C., Silverman,E.K., Everett,S.E. and Stoller,J.K. alpha1-antitrypsin augmentation therapy for PI*MZ heterozygotes: a cautionary note Chest (2008) 134, 831-834. PubMed Europe PubMed DOI

2007

Baek,J.H., Im,H., Kang,U.B., Seong,K.M., Lee,C., Kim,J. and Yu,M.H. Probing the local conformational change of alpha1-antitrypsin Protein Sci (2007) 16, 1842-1850. PubMed Europe PubMed DOI PMC EPMC
Chowdhury,P., Wang,W., Lavender,S., Bunagan,M.R., Klemke,J.W., Tang,J., Saven,J.G., Cooperman,B.S. and Gai,F. Fluorescence correlation spectroscopic study of serpin depolymerization by computationally designed peptides J Mol Biol (2007) 369, 462-473. PubMed Europe PubMed DOI PMC EPMC
Cruz,P.E., Mueller,C. and Flotte,T.R. The promise of gene therapy for the treatment of alpha-1 antitrypsin deficiency Pharmacogenomics (2007) 8, 1191-1198. PubMed Europe PubMed DOI
Kaczor,M.P., Sanak,M. and Szczeklik,A. Rapid and inexpensive detection of alpha1-antitrypsin deficiency-related alleles S and Z by a real-time polymerase chain reaction suitable for a large-scale population-based screening J Mol Diagn (2007) 9, 99-104. PubMed Europe PubMed DOI PMC EPMC
Karnaukhova,E., Ophir,Y., Trinh,L., Dalal,N., Punt,P.J., Golding,B. and Shiloach,J. Expression of human alpha1-proteinase inhibitor in Aspergillus niger Microb Cell Fact (2007) 6, 34-34. PubMed Europe PubMed DOI PMC EPMC E
Liu,L., Mushero,N., Hedstrom,L. and Gershenson,A. Short-lived protease serpin complexes: Partial disruption of the rat trypsin active site Protein Sci (2007) 16, 2403-2411. PubMed Europe PubMed DOI PMC EPMC
Mordwinkin,N.M. and Louie,S.G. Aralast: an alpha1-protease inhibitor for the treatment of alpha-antitrypsin deficiency Expert Opin Pharmacother (2007) 8, 2609-2614. PubMed Europe PubMed DOI U
Mulgrew,A.T., Taggart,C.C. and McElvaney,N.G. Alpha-1-antitrypsin deficiency: current concepts Lung (2007) 185, 191-201. PubMed Europe PubMed DOI M V
Tsuji,A., Kanie,H., Makise,H., Yuasa,K., Nagahama,M. and Matsuda,Y. Engineering of alpha1-antitrypsin variants selective for subtilisin-like proprotein convertases PACE4 and PC6: importance of the P2' residue in stable complex formation of the serpin with proprotein convertase Protein Eng Des Sel (2007) 20, 163-170. PubMed Europe PubMed DOI
Tsutsui,Y. and Wintrode,P.L. Cooperative unfolding of a metastable serpin to a molten globule suggests a link between functional and folding energy landscapes J Mol Biol (2007) 371, 245-255. PubMed Europe PubMed DOI
Weber,A., Engelmaier,A., Owen,M.C., Schwarz,H.P. and Turecek,P.L. Convenient high-resolution isoelectric focusing (IEF) method for the separation of alpha1-proteinase inhibitor (A1PI) isoforms in A1PI concentrates J Pharm Biomed Anal (2007) 45, 107-111. PubMed Europe PubMed DOI
Wood,A.M. and Stockley,R.A. Alpha one antitrypsin deficiency: from gene to treatment Respiration (2007) 74, 481-492. PubMed Europe PubMed DOI V

2006

Chang,Y.P., Mahadeva,R., Chang,W.S., Shukla,A., Dafforn,T.R. and Chu,Y.H. Identification of a 4-mer peptide inhibitor that effectively blocks the polymerization of pathogenic Z alpha1-antitrypsin Am J Respir Cell Mol Biol (2006) 35, 540-548. PubMed Europe PubMed DOI
Dementiev,A., Dobo,J. and Gettins,P.G. Active site distortion is sufficient for proteinase inhibition by serpins: structure of the covalent complex of alpha1-proteinase inhibitor with porcine pancreatic elastase J Biol Chem (2006) 281, 3452-3457. PubMed Europe PubMed DOI S
Karnaukhova,E., Ophir,Y. and Golding,B. Recombinant human alpha-1 proteinase inhibitor: towards therapeutic use Amino Acids (2006) 30, 317-332. PubMed Europe PubMed DOI E
Kolarich,D., Turecek,P.L., Weber,A., Mitterer,A., Graninger,M., Matthiessen,P., Nicolaes,G.A., Altmann,F. and Schwarz,H.P. Biochemical, molecular characterization, and glycoproteomic analyses of alpha(1)-proteinase inhibitor products used for replacement therapy Transfusion (2006) 46, 1959-1977. PubMed Europe PubMed DOI U
[YEAR:15-5-2006]Lomas,D.A. The selective advantage of alpha1-antitrypsin deficiency Am J Respir Crit Care Med (15-5-2006) 173, 1072-1077. PubMed Europe PubMed DOI
Martin,S.L., Downey,D., Bilton,D., Keogan,M.T., Edgar,J. and Elborn,J.S. Safety and efficacy of recombinant alpha(1)-antitrypsin therapy in cystic fibrosis Pediatr Pulmonol (2006) 41, 177-183. PubMed Europe PubMed DOI
Pemberton,P.A., Kobayashi,D., Wilk,B.J., Henstrand,J.M., Shapiro,S.D. and Barr,P.J. Inhaled recombinant alpha 1-antitrypsin ameliorates cigarette smoke-induced emphysema in the mouse COPD (2006) 3, 101-108. PubMed Europe PubMed DOI
Stocks,J.M., Brantly,M., Pollock,D., Barker,A., Kueppers,F., Strange,C., Donohue,J.F. and Sandhaus,R. Multi-center study: the biochemical efficacy, safety and tolerability of a new alpha1-proteinase inhibitor, Zemaira COPD (2006) 3, 17-23. PubMed Europe PubMed DOI
[YEAR:26-9-2006]Sutherland,J.S., Bhakta,V., Filion,M.L. and Sheffield,W.P. The transferable tail: fusion of the N-terminal acidic extension of heparin cofactor II to alpha1-proteinase inhibitor M358R specifically increases the rate of thrombin inhibition Biochemistry (26-9-2006) 45, 11444-11452. PubMed Europe PubMed DOI
[YEAR:30-5-2006]Tsutsui,Y., Liu,L., Gershenson,A. and Wintrode,P.L. The conformational dynamics of a metastable serpin studied by hydrogen exchange and mass spectrometry Biochemistry (30-5-2006) 45, 6561-6569. PubMed Europe PubMed DOI
Zimmerman,T.P. Yield improvement for manufacture of alpha-proteinase inhibitor Vox Sang (2006) 91, 309-315. PubMed Europe PubMed DOI E

2005

Abboud,R.T., Ford,G.T. and Chapman,K.R. Emphysema in alpha1-antitrypsin deficiency: does replacement therapy affect outcome? Treat Respir Med (2005) 4, 1-8. PubMed Europe PubMed DOI
Khatib,H. Monoallelic expression of the protease inhibitor gene in humans, sheep, and cattle Mamm Genome (2005) 16, 50-58. PubMed Europe PubMed DOI M
Louie,S.G., Sclar,D.A. and Gill,M.A. Aralast: a new alpha1-protease inhibitor for treatment of alpha-antitrypsin deficiency Ann Pharmacother (2005) 39, 1861-1869. PubMed Europe PubMed DOI
[YEAR:31-1-2005]Nita,I., Hollander,C., Westin,U. and Janciauskiene,S.M. Prolastin, a pharmaceutical preparation of purified human alpha1-antitrypsin, blocks endotoxin-mediated cytokine release Respir Res (31-1-2005) 6, 12-12. PubMed Europe PubMed DOI PMC EPMC
[YEAR:10-6-2005]Ray,S., Mickleborough,T.D. and Brown,J.L. Comparison of the properties of rare variants of alpha1-proteinase inhibitor expressed in COS-1 cells and assessment of their potential as risk factors in human disease Biochim Biophys Acta Mol Basis Dis (10-6-2005) 1740, 390-402. PubMed Europe PubMed DOI M

2004

Blanco,I., Canto,H., de Serres,F.J., Fernandez-Bustillo,E. and Rodriguez,M.C. Alpha1-antitrypsin replacement therapy controls fibromyalgia symptoms in 2 patients with PI ZZ alpha1-antitrypsin deficiency J Rheumatol (2004) 31, 2082-2085. PubMed Europe PubMed
Carrell,R.W. What we owe to alpha(1)-antitrypsin and to Carl-Bertil Laurell COPD (2004) 1, 71-84. PubMed Europe PubMed DOI V
Chappell,S., Guetta-Baranes,T., Batowski,K., Yiannakis,E., Morgan,K., O'Connor,C., MacNee,W. and Kalsheker,N. Haplotypes of the alpha-1 antitrypsin gene in healthy controls and Z deficiency patients Hum Mutat (2004) 24, 535-536. PubMed Europe PubMed DOI M
Crowther,D.C., Belorgey,D., Miranda,E., Kinghorn,K.J., Sharp,L.K. and Lomas,D.A. Practical genetics: alpha-1-antitrypsin deficiency and the serpinopathies Eur J Hum Genet (2004) 12, 167-172. PubMed Europe PubMed DOI M
Dobo,J. and Gettins,P.G. alpha1-Proteinase inhibitor forms initial non-covalent and final covalent complexes with elastase analogously to other serpin-proteinase pairs, suggesting a common mechanism of inhibition J Biol Chem (2004) 279, 9264-9269. PubMed Europe PubMed DOI
[YEAR:23-11-2004]Filion,M.L., Bhakta,V., Nguyen,L.H., Liaw,P.S. and Sheffield,W.P. Full or partial substitution of the reactive center loop of alpha-1-proteinase inhibitor by that of heparin cofactor II: P1 Arg is required for maximal thrombin inhibition Biochemistry (23-11-2004) 43, 14864-14872. PubMed Europe PubMed DOI
[YEAR:17-12-2004]Jung,C.H., Chae,Y.K. and Im,H. Suppression of the facile latency transition of alpha(1)-antitrypsin variant M(malton) by stabilizing mutations Biochem Biophys Res Commun (17-12-2004) 325, 744-750. PubMed Europe PubMed DOI
Jung,C.H., Na,Y.R. and Im,H. Retarded protein folding of deficient human alpha 1-antitrypsin D256V and L41P variants Protein Sci (2004) 13, 694-702. PubMed Europe PubMed DOI PMC EPMC
[YEAR:15-10-2004]Kang,U.B., Baek,J.H., Ryu,S.H., Kim,J., Yu,M.H. and Lee,C. Kinetic mechanism of protease inhibition by alpha1-antitrypsin Biochem Biophys Res Commun (15-10-2004) 323, 409-415. PubMed Europe PubMed DOI
[YEAR:1-12-2004]Li,W., Savinov,A.Y., Rozanov,D.V., Golubkov,V.S., Hedayat,H., Postnova,T.I., Golubkova,N.V., Linli,Y., Krajewski,S. and Strongin,A.Y. Matrix metalloproteinase-26 is associated with estrogen-dependent malignancies and targets alpha1-antitrypsin serpin Cancer Res (1-12-2004) 64, 8657-8665. PubMed Europe PubMed DOI
Luisetti,M. and Seersholm,N. Alpha1-antitrypsin deficiency. 1: Epidemiology of alpha1-antitrypsin deficiency Thorax (2004) 59, 164-169. PubMed Europe PubMed DOI PMC EPMC
Mulgrew,A.T., Taggart,C.C., Lawless,M.W., Greene,C.M., Brantly,M.L., O'Neill,S.J. and McElvaney,N.G. Z alpha1-antitrypsin polymerizes in the lung and acts as a neutrophil chemoattractant Chest (2004) 125, 1952-1957. PubMed Europe PubMed DOI M
[YEAR:10-6-2004]Nie,J. and Pei,D. Rapid inactivation of alpha-1-proteinase inhibitor by neutrophil specific leukolysin/membrane-type matrix metalloproteinase 6 Exp Cell Res (10-6-2004) 296, 145-150. PubMed Europe PubMed DOI
Parfrey,H., Dafforn,T.R., Belorgey,D., Lomas,D.A. and Mahadeva,R. Inhibiting polymerization: new therapeutic strategies for Z alpha1-antitrypsin-related emphysema Am J Respir Cell Mol Biol (2004) 31, 133-139. PubMed Europe PubMed DOI
[YEAR:16-2-2004]Pullikotil,P., Vincent,M., Nichol,S.T. and Seidah,N.G. Development of protein-based inhibitors of the proprotein of convertase SKI-1/S1P: processing of SREBP-2, ATF6, and a viral glycoprotein J Biol Chem (16-2-2004) 279, 17338-17347. PubMed Europe PubMed DOI

2003

[YEAR:15-2-2003]Bousquet,J.A., Duranton,J., Mely,Y. and Bieth,J.G. Conformational change in elastase following complexation with alpha1-proteinase inhibitor: a CD investigation Biochem J (15-2-2003) 370, 345-349. PubMed Europe PubMed DOI PMC EPMC
de Serres,F.J. Alpha-1 antitrypsin deficiency is not a rare disease but a disease that is rarely diagnosed Environ Health Perspect (2003) 111, 1851-1854. PubMed Europe PubMed PMC EPMC
[YEAR:26-9-2003]Dementiev,A., Simonovic,M., Volz,K. and Gettins,P.G. Canonical inhibitor-like interactions explain reactivity of alpha1-proteinase inhibitor Pittsburgh and antithrombin with proteinases J Biol Chem (26-9-2003) 278, 37881-37887. PubMed Europe PubMed DOI M S
Duranton,J. and Bieth,J.G. Inhibition of proteinase 3 by [alpha]1-antitrypsin in vitro predicts very fast inhibition in vivo Am J Respir Cell Mol Biol (2003) 29, 57-61. PubMed Europe PubMed DOI
Forsyth,S., Horvath,A. and Coughlin,P. A review and comparison of the murine alpha1-antitrypsin and alpha1-antichymotrypsin multigene clusters with the human clade A serpins Genomics (2003) 81, 336-345. PubMed Europe PubMed DOI
[YEAR:30-5-2003]Marszal,E., Danino,D. and Shrake,A. A novel mode of polymerization of alpha1-proteinase inhibitor J Biol Chem (30-5-2003) 278, 19611-19618. PubMed Europe PubMed DOI M
[YEAR:29-8-2003]Parfrey,H., Mahadeva,R., Ravenhill,N.A., Zhou,A., Dafforn,T.R., Foreman,R.C. and Lomas,D.A. Targeting a surface cavity of alpha 1-antitrypsin to prevent conformational disease J Biol Chem (29-8-2003) 278, 33060-33066. PubMed Europe PubMed DOI M
[YEAR:27-2-2003]Wilczynska,M., Lobov,S. and Ny,T. The spontaneous polymerization of plasminogen activator inhibitor type-2 and Z-antitrypsin are due to different molecular aberrations FEBS Lett (27-2-2003) 537, 11-16. PubMed Europe PubMed DOI M

2002

Barbour,K.W., Goodwin,R.L., Guillonneau,F., Wang,Y., Baumann,H. and Berger,F.G. Functional diversification during evolution of the murine alpha(1)-proteinase inhibitor family: role of the hypervariable reactive center loop Mol Biol Evol (2002) 19, 718-727. PubMed Europe PubMed
Barbour,K.W., Wei,F., Brannan,C., Flotte,T.R., Baumann,H. and Berger,F.G. The murine alpha(1)-proteinase inhibitor gene family: polymorphism, chromosomal location, and structure Genomics (2002) 80, 515-522. PubMed Europe PubMed DOI M
Cantin,A.M., Woods,D.E., Cloutier,D., Dufour,E.K. and Leduc,R. Polyethylene glycol conjugation at Cys232 prolongs the half-life of alpha1 proteinase inhibitor Am J Respir Cell Mol Biol (2002) 27, 659-665. PubMed Europe PubMed DOI
Chowanadisai,W. and Lonnerdal,B. Alpha(1)-antitrypsin and antichymotrypsin in human milk: origin, concentrations, and stability Am J Clin Nutr (2002) 76, 828-833. PubMed Europe PubMed
Im,H., Woo,M.S., Hwang,K.Y. and Yu,M.H. Interactions causing the kinetic trap in serpin protein folding J Biol Chem (2002) 277, 46347-46354. PubMed Europe PubMed DOI S
[YEAR:16-4-2002]Irving,J.A., Pike,R.N., Dai,W., Bromme,D., Worrall,D.M., Silverman,G.A., Coetzer,T.H., Dennison,C., Bottomley,S.P. and Whisstock,J.C. Evidence that serpin architecture intrinsically supports papain-like cysteine protease inhibition: engineering alpha(1)-antitrypsin to inhibit cathepsin proteases Biochemistry (16-4-2002) 41, 4998-5004. PubMed Europe PubMed DOI
Lomas,D.A., Lourbakos,A., Cumming,S.A. and Belorgey,D. Hypersensitive mousetraps, alpha1-antitrypsin deficiency and dementia Biochem Soc Trans (2002) 30, 89-92. PubMed Europe PubMed
[YEAR:1-3-2002]Mahadeva,R., Dafforn,T.R., Carrell,R.W. and Lomas,D.A. 6-mer peptide selectively anneals to a pathogenic serpin conformation and blocks polymerization. Implications for the prevention of Z alpha(1)-antitrypsin-related cirrhosis J Biol Chem (1-3-2002) 277, 6771-6774. PubMed Europe PubMed DOI
[YEAR:11-10-2002]Mellet,P., Mely,Y., Hedstrom,L., Cahoon,M., Belorgey,D., Srividya,N., Rubin,H. and Bieth,J.G. Comparative trajectories of active and S195A inactive trypsin upon binding to serpins J Biol Chem (11-10-2002) 277, 38901-38914. PubMed Europe PubMed DOI
[YEAR:15-9-2002]Rodriguez,F., Jardi,R., Costa,X., Cotrina,M., Galimany,R., Vidal,R. and Miravitlles,M. Rapid screening for alpha1-antitrypsin deficiency in patients with chronic obstructive pulmonary disease using dried blood specimens Am J Respir Crit Care Med (15-9-2002) 166, 814-817. PubMed Europe PubMed DOI
Sandoval,C., Curtis,H. and Congote,L.F. Enhanced proliferative effects of a baculovirus-produced fusion protein of insulin-like growth factor and alpha(1)-proteinase inhibitor and improved anti-elastase activity of the inhibitor with glutamate at position 351 Protein Eng (2002) 15, 413-418. PubMed Europe PubMed
Stoller,J.K., Rouhani,F., Brantly,M., Shahin,S., Dweik,R.A., Stocks,J.M., Clausen,J., Campbell,E. and Norton,F. Biochemical efficacy and safety of a new pooled human plasma alpha(1)-antitrypsin, Respitin Chest (2002) 122, 66-74. PubMed Europe PubMed DOI
Sulikowski,T., Bauer,B.A. and Patston,P.A. alpha(1)-Proteinase inhibitor mutants with specificity for plasma kallikrein and C1s but not C1 Protein Sci (2002) 11, 2230-2236. PubMed Europe PubMed DOI PMC EPMC
Tsuji,A., Ikoma,T., Hashimoto,E. and Matsuda,Y. Development of selectivity of alpha1-antitrypsin variant by mutagenesis in its reactive site loop against proprotein convertase. A crucial role of the P4 arginine in PACE4 inhibition Protein Eng (2002) 15, 123-130. PubMed Europe PubMed

2001

Gladysheva,I.P., Popykina,N.A., Zamolodchikova,T.S. and Larionova,N.I. Interaction between duodenase and alpha1-proteinase inhibitor Biochemistry (Mosc) (2001) 66, 682-687. PubMed Europe PubMed DOI
Kim,S., Woo,J., Seo,E.J., Yu,M. and Ryu,S. A 2.1 A resolution structure of an uncleaved alpha(1)-antitrypsin shows variability of the reactive center and other loops J Mol Biol (2001) 306, 109-119. PubMed Europe PubMed DOI S
Kropp,J., Wencker,M., Hotze,A., Banik,N., Hubner,G.E., Wunderlich,G., Ulbrich,E., Konietzko,N. and Biersack,H.J. Inhalation of [123I]alpha1-protease inhibitor: Toward a new therapeutic concept of alpha1-protease inhibitor deficiency? J Nucl Med (2001) 42, 744-751. PubMed Europe PubMed
[YEAR:29-5-2001]Peterson,F.C. and Gettins,P.G. Insight into the mechanism of serpin-proteinase inhibition from 2D [1H-15N] NMR studies of the 69 kDa alpha1-proteinase inhibitor Pittsburgh-trypsin covalent complex Biochemistry (29-5-2001) 40, 6284-6292. PubMed Europe PubMed DOI
[YEAR:6-4-2001]Tew,D.J. and Bottomley,S.P. Intrinsic fluorescence changes and rapid kinetics of proteinase deformation during serpin inhibition FEBS Lett (6-4-2001) 494, 30-33. PubMed Europe PubMed DOI

2000

Dunstone,M.A., Dai,W., Whisstock,J.C., Rossjohn,J., Pike,R.N., Feil,S.C., le Bonniec,B.F., Parker,M.W. and Bottomley,S.P. Cleaved antitrypsin polymers at atomic resolution Protein Sci (2000) 9, 417-420. PubMed Europe PubMed DOI PMC EPMC S
[YEAR:3-10-2000]Peterson,F.C., Gordon,N.C. and Gettins,P.G. Formation of a noncovalent serpin-proteinase complex involves no conformational change in the serpin. Use of 1H-15N HSQC NMR as a sensitive nonperturbing monitor of conformation Biochemistry (3-10-2000) 39, 11884-11892. PubMed Europe PubMed DOI

1999

Huntington,J.A., Pannu,N.S., Hazes,B., Read,R.J., Lomas,D.A. and Carrell,R.W. A 2.6 A structure of a serpin polymer and implications for conformational disease J Mol Biol (1999) 293, 449-455. PubMed Europe PubMed DOI S
Rapala-Kozik,M., Potempa,J., Nelson,D., Kozik,A. and Travis,J. Comparative cleavage sites within the reactive-site loop of native and oxidized alpha1-proteinase inhibitor by selected bacterial proteinases Biol Chem (1999) 380, 1211-1216. PubMed Europe PubMed DOI
Tsuji,A., Hashimoto,E., Ikoma,T., Taniguchi,T., Mori,K., Nagahama,M. and Matsuda,Y. Inactivation of proprotein convertase, PACE4, by alpha1-antitrypsin Portland (alpha1-PDX), a blocker of proteolytic activation of bone morphogenetic protein during embryogenesis: evidence that PACE4 is able to form an SDS-stable acyl intermediate with alpha1-PDX J Biochem (1999) 126, 591-603. PubMed Europe PubMed I

1998

[YEAR:10-4-1998]Dufour,E.K., Denault,J.B., Hopkins,P.C. and Leduc,R. Serpin-like properties of alpha1-antitrypsin Portland towards furin convertase FEBS Lett (10-4-1998) 426, 41-46. PubMed Europe PubMed
[YEAR:23-1-1998]Elliott,P.R., Abrahams,J.P. and Lomas,D.A. Wild-type alpha 1-antitrypsin is in the canonical inhibitory conformation J Mol Biol (23-1-1998) 275, 419-425. PubMed Europe PubMed DOI
Jean,F., Stella,K., Thomas,L., Liu,G., Xiang,Y., Reason,A.J. and Thomas,G. alpha1-Antitrypsin Portland, a bioengineered serpin highly selective for furin: application as an antipathogenic agent Proc Natl Acad Sci U S A (1998) 95, 7293-7298. PubMed Europe PubMed DOI PMC EPMC M I

1997

Cook,L., Knight,K.R., Burdon,J.G.W., Brenton,S. and Hunt,J.M. Function of a rare variant of alpha-1-antitrypsin, phenotype Pi E(Franklin) S, a poor inhibitor of human neutrophil elastase Res Exp Med (Berl) (1997) 196, 355-362. PubMed Europe PubMed DOI M
Kaslik,G., Kardos,J., Szabo,E., Szilagyi,L., Zavodszky,P., Westler,W.M., Markley,J.L. and Graf,L. Effects of serpin binding on the target proteinase: global stabilization, localized increased structural flexibility, and conserved hydrogen bonding at the active site Biochemistry (1997) 36, 5455-5464. PubMed Europe PubMed DOI
Mains,R.E., Berard,C.A., Denault,J.B., Zhou,A., Johnson,R.C. and Leduc,R. PACE4: a subtilisin-like endoprotease with unique properties Biochem J (1997) 321, 587-593. PubMed Europe PubMed PMC EPMC I

1996

Elliott,P.R., Lomas,D.A., Carrell,R.W. and Abrahams,J.P. Inhibitory conformation of the reactive loop of alpha 1-antitrypsin Nat Struct Biol (1996) 3, 676-681. PubMed Europe PubMed DOI S
Goodwin,R.L., Baumann,H. and Berger,F.G. Patterns of divergence during evolution of alpha 1-proteinase inhibitors in mammals Mol Biol Evol (1996) 13, 346-358. PubMed Europe PubMed
Ryu,S.E., Choi,H.J., Kwon,K.S., Lee,K.N. and Yu,M.H. The native strains in the hydrophobic core and flexible reactive loop of a serine protease inhibitor: crystal structure of an uncleaved alpha1-antitrypsin at 2.7 A Structure (1996) 4, 1181-1192. PubMed Europe PubMed S

1995

Song,H.K., Lee,K.N., Kwon,K.S., Yu,M.H. and Suh,S.W. Crystal structure of an uncleaved alpha 1-antitrypsin reveals the conformation of its inhibitory reactive loop FEBS Lett (1995) 377, 150-154. PubMed Europe PubMed DOI S

1994

Sires,U.I., Murphy,G., Baragi,V.M., Fliszar,C.J., Welgus,H.G. and Senior,R.M. Matrilysin is much more efficient than other matrix metalloproteinases in the proteolytic inactivation of alpha1-antitrypsin Biochem Biophys Res Commun (1994) 204, 613-620. PubMed Europe PubMed DOI
[YEAR:2-3-1994]Zhang,Z., Winyard,P.G., Chidwick,K., Murphy,G., Wardell,M., Carrell,R.W. and Blake,D.R. Proteolysis of human native and oxidised alpha 1-proteinase inhibitor by matrilysin and stromelysin Biochim Biophys Acta (2-3-1994) 1199, 224-228. PubMed Europe PubMed DOI

1993

Stoller,J.K., Hupertz,V. and Aboussouan,L.S. Alpha-1 Antitrypsin Deficiency (1993) PubMed Europe PubMed

1992

Chao,J., Chen,L.M., Chai,K.X. and Chao,L. Expression of kallikrein-binding protein and alpha 1-antitrypsin genes in response to sex hormones, growth, inflammation and hypertension Agents Actions Suppl (1992) 38, 174-181. PubMed Europe PubMed
Imamura,T. and Kambara,T. The major plasma kallikrein inhibitor of guinea pig plasma Biochim Biophys Acta (1992) 1118, 261-266. PubMed Europe PubMed DOI
Mast,A.E., Enghild,J.J. and Salvesen,G. Conformation of the reactive site loop of alpha 1-proteinase inhibitor probed by limited proteolysis Biochemistry (1992) 31, 2720-2728. PubMed Europe PubMed P
Michaelis,J., Vissers,M.C. and Winterbourn,C.C. Cleavage of alpha 1-antitrypsin by human neutrophil collagenase Matrix Suppl (1992) 1, 80-81. PubMed Europe PubMed
Schulze,A.J., Frohnert,P.W., Engh,R.A. and Huber,R. Evidence for the extent of insertion of the active site loop of intact alpha1 proteinase inhibitor in beta-sheet A Biochemistry (1992) 31, 7560-7565. PubMed Europe PubMed
[YEAR:14-9-1992]Serveau,C., Moreau,T., Zhou,G.X., ElMoujahed,A., Chao,J. and Gauthier,F. Inhibition of rat tissue kallikrein gene family members by rat kallikrein-binding protein and alpha 1-proteinase inhibitor FEBS Lett (14-9-1992) 309, 405-408. PubMed Europe PubMed DOI
Suzuki,Y. and Sinohara,H. Molecular evolution and acute phase change of guinea pig contrapsin and alpha-1-antiproteinase Folia Histochem Cytobiol (1992) 30, 191-193. PubMed Europe PubMed

1991

Frommherz,K.J., Faller,B. and Bieth,J.G. Heparin strongly decreases the rate of inhibition of neutrophil elastase by alpha1-proteinase inhibitor J Biol Chem (1991) 266, 15356-15362. PubMed Europe PubMed
Mast,A.E., Enghild,J.J., Nagase,H., Suzuki,K., Pizzo,S.V. and Salvesen,G. Kinetics and physiologic relevance of the inactivation of alpha1- proteinase inhibitor, alpha1-antichymotrypsin, and antithrombin III by matrix metalloproteinases-1 (tissue collagenase), -2 (72-kDa gelatinase/type IV collagenase), and -3 (stromelysin) J Biol Chem (1991) 266, 15810-15816. PubMed Europe PubMed
Schulze,A.J., Huber,R., Degryse,E., Speck,D. and Bischoff,R. Inhibitory activity and conformational transition of alpha1-proteinase inhibitor variants Eur J Biochem (1991) 202, 1147-1155. PubMed Europe PubMed S
[YEAR:15-1-1991]Suzuki,Y., Yoshida,K., Honda,E. and Sinohara,H. Molecular cloning and sequence analysis of cDNAs coding for guinea pig alpha 1-antiproteinases S and F and contrapsin J Biol Chem (15-1-1991) 266, 928-932. PubMed Europe PubMed

1990

[YEAR:24-4-1990]Knauper,V., Reinke,H. and Tschesche,H. Inactivation of human plasma alpha1-proteinase inhibitor by human PMN leucocyte collagenase FEBS Lett (24-4-1990) 263, 355-357. PubMed Europe PubMed DOI
[YEAR:31-8-1990]Misumi,Y., Ohkubo,K., Sohda,M., Takami,N., Oda,K. and Ikehara,Y. Intracellular processing of complement pro-C3 and proalbumin is inhibited by rat alpha 1-protease inhibitor variant (Met352-->Arg) in transfected cells Biochem Biophys Res Commun (31-8-1990) 171, 236-242. PubMed Europe PubMed DOI
Suzuki,Y., Yoshida,K., Ichimiya,T., Yamamoto,T. and Sinohara,H. Trypsin inhibitors in guinea pig plasma: isolation and characterization of contrapsin and two isoforms of alpha-1-antiproteinase and acute phase response of four major trypsin inhibitors J Biochem (1990) 107, 173-179. PubMed Europe PubMed

1989

Engh,R., Lobermann,H., Schneider,M., Wiegand,G., Huber,R. and Laurell,C.B. The S variant of human alpha1-antitrypsin, structure and implications for function and metabolism Protein Eng (1989) 2, 407-415. PubMed Europe PubMed S
Huber,R. and Carrell,R.W. Implications of the three-dimensional structure of alpha1- antitrypsin for structure and function of serpins Biochemistry (1989) 28, 8951-8966. PubMed Europe PubMed S

1988

Banda,M.J., Rice,A.G., Griffin,G.L. and Senior,R.M. Alpha 1-proteinase inhibitor is a neutrophil chemoattractant after proteolytic inactivation by macrophage elastase J Biol Chem (1988) 263, 4481-4484. PubMed Europe PubMed
Conlan,J.W., Williams,A. and Ashworth,L.A. Inactivation of human alpha-1-antitrypsin by a tissue-destructive protease of Legionella pneumophila J Gen Microbiol (1988) 134, 481-487. PubMed Europe PubMed

1987

Banda,M.J., Clark,E.J., Sinha,S. and Travis,J. Interaction of mouse macrophage elastase with native and oxidized human alpha 1-proteinase inhibitor J Clin Invest (1987) 79, 1314-1317. PubMed Europe PubMed DOI PMC EPMC

1986

Heidtmann,H. and Travis,J. Human alpha1-proteinase inhibitor [ISSN:0-444-80763-2] (1986) , 441-456.
Johnson,D.A., Barrett,A.J. and Mason,R.W. Cathepsin L inactivates alpha1-proteinase inhibitor by cleavage in the reactive site region J Biol Chem (1986) 261, 14748-14751. PubMed Europe PubMed
Schapira,M., Ramus,M.A., Jallat,S., Carvallo,D. and Courtney,M. Recombinant alpha1-antitrypsin Pittsburgh (Met 358->Arg) is a potent inhibitor of plasma kallikrein and activated factor XII fragment J Clin Invest (1986) 77, 635-637. PubMed Europe PubMed DOI PMC EPMC M
Scott,C.F., Carrell,R.W., Glaser,C.B., Kueppers,F., Lewis,J.H. and Colman,R.W. Alpha-1-antitrypsin-Pittsburgh. A potent inhibitor of human plasma factor XIa, kallikrein, and factor XIIf J Clin Invest (1986) 77, 631-634. PubMed Europe PubMed DOI PMC EPMC

1985

[YEAR:24-10-1985]Carrell,R.W. and Owen,M.C. Plakalbumin, alpha 1-antitrypsin, antithrombin and the mechanism of inflammatory thrombosis Nature (24-10-1985) 317, 730-732. PubMed Europe PubMed
[YEAR:10-1-1985]Courtney,M., Jallat,S., Tessier,L.H., Benavente,A., Crystal,R.G. and Lecocq,J.P. Synthesis in E. coli of alpha 1-antitrypsin variants of therapeutic potential for emphysema and thrombosis Nature (10-1-1985) 313, 149-151. PubMed Europe PubMed E
[YEAR:16-1-1985]Laurent,P. and Bieth,J.G. Cigarette smoke decreases the rate constant for the association of elastase with alpha 1-proteinase inhibitor by a non-oxidative mechanism Biochem Biophys Res Commun (16-1-1985) 126, 275-281. PubMed Europe PubMed
[YEAR:10-4-1985]Travis,J., Owen,M., George,P., Carrell,R., Rosenberg,S., Hallewell,R.A. and Barr,P.J. Isolation and properties of recombinant DNA produced variants of human alpha 1-proteinase inhibitor J Biol Chem (10-4-1985) 260, 4384-4389. PubMed Europe PubMed

1984

[YEAR:19-11-1984]Bathurst,I.C., Travis,J., George,P.M. and Carrell,R.W. Structural and functional characterization of the abnormal Z alpha 1-antitrypsin isolated from human liver FEBS Lett (19-11-1984) 177, 179-183. PubMed Europe PubMed DOI
Beatty,K., Matheson,N. and Travis,J. Kinetic and chemical evidence for the inability of oxidized alpha 1-proteinase inhibitor to protect lung elastin from elastolytic degradation Hoppe Seylers Z Physiol Chem (1984) 365, 731-736. PubMed Europe PubMed
Bruce,R.M., Cohen,B.H., Diamond,E.L., Fallat,R.J., Knudson,R.J., Lebowitz,M.D., Mittman,C., Patterson,C.D. and Tockman,M.S. Collaborative study to assess risk of lung disease in Pi MZ phenotype subjects Am Rev Respir Dis (1984) 130, 386-390. PubMed Europe PubMed
Cox,D.W. and Billingsley,G.D. Oxidation of plasma alpha 1-antitrypsin in smokers and nonsmokers and by an oxidizing agent Am Rev Respir Dis (1984) 130, 594-599. PubMed Europe PubMed
[YEAR:23-6-1984]Eriksson,S. Alpha 1-antitrypsin deficiency: some personal experiences Schweiz Med Wochenschr (23-6-1984) 114, 893-894. PubMed Europe PubMed
[YEAR:12-3-1984]Foreman,R.C., Judah,J.D. and Colman,A. Xenopus oocytes can synthesise but do not secrete the Z variant of human alpha 1-antitrypsin FEBS Lett (12-3-1984) 168, 84-88. PubMed Europe PubMed
Fuchs,H.E., Michalopoulos,G.K. and Pizzo,S.V. Hepatocyte uptake of alpha 1-proteinase inhibitor-trypsin complexes in vitro: evidence for a shared uptake mechanism for proteinase complexes of alpha 1-proteinase inhibitor and antithrombin III J Cell Biochem (1984) 25, 231-243. PubMed Europe PubMed DOI
[YEAR:22-12-1984]George,P.M., Vissers,M.C., Travis,J., Winterbourn,C.C. and Carrell,R.W. A genetically engineered mutant of alpha 1-antitrypsin protects connective tissue from neutrophil damage and may be useful in lung disease Lancet (22-12-1984) 2, 1426-1428. PubMed Europe PubMed
[YEAR:10-6-1984]Gitel,S.N., Medina,V.M. and Wessler,S. Inhibition of human activated factor X by antithrombin III and alpha 1-proteinase inhibitor in human plasma J Biol Chem (10-6-1984) 259, 6890-6895. PubMed Europe PubMed
[YEAR:13-9-1984]Hill,R.E., Shaw,P.H., Boyd,P.A., Baumann,H. and Hastie,N.D. Plasma protease inhibitors in mouse and man: divergence within the reactive centre regions Nature (13-9-1984) 311, 175-177. PubMed Europe PubMed
Hirano,K., Okumura,Y., Hayakawa,S., Adachi,T. and Sugiura,M. Inhibition of human tissue kallikrein by alpha 1-proteinase inhibitor Hoppe Seylers Z Physiol Chem (1984) 365, 27-32. PubMed Europe PubMed
[YEAR:8-3-1984]Kidd,V.J., Golbus,M.S., Wallace,R.B., Itakura,K. and Woo,S.L. Prenatal diagnosis of alpha 1-antitrypsin deficiency by direct analysis of the mutation site in the gene N Engl J Med (8-3-1984) 310, 639-642. PubMed Europe PubMed DOI
Loebermann,H., Tokuoka,R., Deisenhofer,J. and Huber,R. Human alpha1-proteinase inhibitor. Crystal structure analysis of two crystal modifications, molecular model and preliminary analysis of the implications for function J Mol Biol (1984) 177, 531-557. PubMed Europe PubMed S
[YEAR:9-10-1984]Long,G.L., Chandra,T., Woo,S.L., Davie,E.W. and Kurachi,K. Complete sequence of the cDNA for human alpha 1-antitrypsin and the gene for the S variant Biochemistry (9-10-1984) 23, 4828-4837. PubMed Europe PubMed
Morihara,K., Tsuzuki,H., Harada,M. and Iwata,T. Purification of human plasma alpha 1-proteinase inhibitor and its inactivation by Pseudomonas aeruginosa elastase J Biochem (1984) 95, 795-804. PubMed Europe PubMed
Neumann,S., Gunzer,G., Hennrich,N. and Lang,H. "PMN-elastase assay": enzyme immunoassay for human polymorphonuclear elastase complexed with alpha 1-proteinase inhibitor J Clin Chem Clin Biochem (1984) 22, 693-697. PubMed Europe PubMed
[YEAR:1-11-1984]Rosenberg,S., Barr,P.J., Najarian,R.C. and Hallewell,R.A. Synthesis in yeast of a functional oxidation-resistant mutant of human alpha-antitrypsin Nature (1-11-1984) 312, 77-80. PubMed Europe PubMed E
[YEAR:10-7-1984]Tanaka,T., Ohkubo,H. and Nakanishi,S. Common structural organization of the angiotensinogen and the alpha 1-antitrypsin genes J Biol Chem (10-7-1984) 259, 8063-8065. PubMed Europe PubMed
[YEAR:23-6-1984]Virca,G.D. and Schnebli,H.P. The elastase/alpha 1-proteinase inhibitor balance in the lung. A review Schweiz Med Wochenschr (23-6-1984) 114, 895-898. PubMed Europe PubMed
Wyss,A., Virca,G.D. and Schnebli,H.P. Cigarette smoke components are not very effective in directly inactivating alpha 1-proteinase inhibitor Hoppe Seylers Z Physiol Chem (1984) 365, 511-516. PubMed Europe PubMed

1983

[YEAR:21-3-1983]Bathurst,I.C., Stenflo,J., Errington,D.M. and Carrell,R.W. Translation and processing of normal (PiMM) and abnormal (PiZZ) human alpha 1-antitrypsin FEBS Lett (21-3-1983) 153, 270-274. PubMed Europe PubMed DOI
[YEAR:28-4-1983]Boswell,D.R., Jeppsson,J.O., Brennan,S.O. and Carrell,R.W. The reactive site of alpha 1-antitrypsin is C-terminal, not N-terminal Biochim Biophys Acta (28-4-1983) 744, 212-218. PubMed Europe PubMed
Carp,H., Janoff,A., Abrams,W., Weinbaum,G., Drew,R.T., Weissbach,H. and Brot,N. Human methionine sulfoxide-peptide reductase, an enzyme capable of reactivating oxidized alpha-1-proteinase inhibitor in vitro Am Rev Respir Dis (1983) 127, 301-305. PubMed Europe PubMed
[YEAR:25-10-1983]Chandra,T., Stackhouse,R., Kidd,V.J., Robson,K.J. and Woo,S.L. Sequence homology between human alpha 1-antichymotrypsin, alpha 1-antitrypsin, and antithrombin III Biochemistry (25-10-1983) 22, 5055-5061. PubMed Europe PubMed
Idell,S. and Cohen,A.B. Alpha-1-antitrypsin deficiency Clin Chest Med (1983) 4, 359-375. PubMed Europe PubMed
[YEAR:21-7-1983]Kidd,V.J., Wallace,R.B., Itakura,K. and Woo,S.L. alpha 1-antitrypsin deficiency detection by direct analysis of the mutation in the gene Nature (21-7-1983) 304, 230-234. PubMed Europe PubMed
Lai,E.C., Kao,F.T., Law,M.L. and Woo,S.L. Assignment of the alpha 1-antitrypsin gene and a sequence-related gene to human chromosome 14 by molecular hybridization Am J Hum Genet (1983) 35, 385-392. PubMed Europe PubMed PMC EPMC
[YEAR:22-9-1983]Owen,M.C., Brennan,S.O., Lewis,J.H. and Carrell,R.W. Mutation of antitrypsin to antithrombin: alpha 1-antitrypsin Pittsburgh (358 Met to Arg), a fatal bleeding disorder N Engl J Med (22-9-1983) 309, 694-698. PubMed Europe PubMed DOI

1982

Carp,H., Miller,F., Hoidal,J.R. and Janoff,A. Potential mechanism of emphysema: alpha 1-proteinase inhibitor recovered from lungs of cigarette smokers contains oxidized methionine and has decreased elastase inhibitory capacity Proc Natl Acad Sci U S A (1982) 79, 2041-2045. PubMed Europe PubMed PMC EPMC
[YEAR:22-7-1982]Carrell,R.W., Jeppsson,J.O., Laurell,C.B., Brennan,S.O., Owen,M.C., Vaughan,L. and Boswell,D.R. Structure and variation of human alpha 1-antitrypsin Nature (22-7-1982) 298, 329-334. PubMed Europe PubMed
[YEAR:1-11-1982]Errington,D.M., Bathurst,I.C., Janus,E.D. and Carrell,R.W. In vitro synthesis of M and Z forms of human alpha 1-antitrypsin FEBS Lett (1-11-1982) 148, 83-86. PubMed Europe PubMed DOI
[YEAR:24-6-1982]Leicht,M., Long,G.L., Chandra,T., Kurachi,K., Kidd,V.J., Mace,M., Jr., Davie,E.W. and Woo,S.L. Sequence homology and structural comparison between the chromosomal human alpha 1-antitrypsin and chicken ovalbumin genes Nature (24-6-1982) 297, 655-659. PubMed Europe PubMed
Lobermann,H., Lottspeich,F., Bode,W. and Huber,R. Interaction of human alpha1-proteinase inhibitor with chymotrypsinogen A and crystallization of a proteolytically modified alpha1-proteinase inhibitor Hoppe Seylers Z Physiol Chem (1982) 363, 1377-1388. PubMed Europe PubMed
Tobin,M.J. and Hutchison,D.C. An overview of the pulmonary features of alpha 1-antitrypsin deficiency Arch Intern Med (1982) 142, 1342-1348. PubMed Europe PubMed DOI M
[YEAR:4-3-1982]Vaughan,L., Lorier,M.A. and Carrell,R.W. alpha 1-Antitrypsin microheterogeneity. Isolation and physiological significance of isoforms Biochim Biophys Acta (4-3-1982) 701, 339-345. PubMed Europe PubMed
[YEAR:4-6-1982]Virca,G.D., Lyerly,D., Kreger,A. and Travis,J. Inactivation of human plasma alpha 1-proteinase inhibitor by a metalloproteinase from Serratia marcescens Biochim Biophys Acta (4-6-1982) 704, 267-271. PubMed Europe PubMed

1981

[YEAR:30-11-1981]Chandra,T., Kurachi,K., Davie,E.W. and Woo,S.L. Induction of alpha 1-antitrypsin mRNA and cloning of its cDNA Biochem Biophys Res Commun (30-11-1981) 103, 751-758. PubMed Europe PubMed
[YEAR:10-4-1981]Clark,R.A., Stone,P.J., El Hag,A., Calore,J.D. and Franzblau,C. Myeloperoxidase-catalyzed inactivation of alpha 1-protease inhibitor by human neutrophils J Biol Chem (10-4-1981) 256, 3348-3353. PubMed Europe PubMed
Gadek,J.E., Klein,H.G., Holland,P.V. and Crystal,R.G. Replacement therapy of alpha 1-antitrypsin deficiency. Reversal of protease-antiprotease imbalance within the alveolar structures of PiZ subjects J Clin Invest (1981) 68, 1158-1165. PubMed Europe PubMed PMC EPMC
Kurachi,K., Chandra,T., Degen,S.J., White,T.T., Marchioro,T.L., Woo,S.L. and Davie,E.W. Cloning and sequence of cDNA coding for alpha1-antitrypsin Proc Natl Acad Sci U S A (1981) 78, 6826-6830. PubMed Europe PubMed PMC EPMC
Travis,J. and Johnson,D. [55] Human alpha1-proteinase inhibitor Methods Enzymol (1981) 80, 754-765. DOI

1980

Banda,M.J., Clark,E.J. and Werb,Z. Limited proteolysis by macrophage elastase inactivates human alpha1-proteinase inhibitor J Exp Med (1980) 152, 1563-1570. PubMed Europe PubMed PMC EPMC
Brodrick,J.W., Glaser,C.B., Largman,C., Geokas,M.C., Graceffo,M., Fassett,M. and Maeda,H. Interaction of chymotrypsinogens with alpha1-protease inhibitor Biochemistry (1980) 19, 4865-4870. PubMed Europe PubMed
Carrell,R.W., Boswell,D.R., Brennan,S.O. and Owen,M.C. Active site of alpha1-antitrypsin: homologous site in antithrombin-III Biochem Biophys Res Commun (1980) 93, 399-402. PubMed Europe PubMed
Mahoney,W.C., Kurachi,K. and Hermodson,M.A. Formation and dissociation of the covalent complexes between trypsin and two homologous inhibitors, alpha 1-antitrypsin and antithrombin III Eur J Biochem (1980) 105, 545-552. PubMed Europe PubMed
[YEAR:10-5-1980]Mega,T., Lujan,E. and Yoshida,A. Studies on the oligosaccharide chains of human alpha 1-protease inhibitor. II. Structure of oligosaccharides J Biol Chem (10-5-1980) 255, 4057-4061. PubMed Europe PubMed
[YEAR:16-10-1980]Wong,P.S. and Travis,J. Isolation and properties of oxidized alpha-1-proteinase inhibitor from human rheumatoid synovial fluid Biochem Biophys Res Commun (16-10-1980) 96, 1449-1454. PubMed Europe PubMed

1979

[YEAR:14-12-1979]Carrell,R., Owen,M., Brennan,S. and Vaughan,L. Carboxy terminal fragment of human alpha-1-antitrypsin from hydroxylamine cleavage: homology with antithrombin III Biochem Biophys Res Commun (14-12-1979) 91, 1032-1037. PubMed Europe PubMed
[YEAR:10-9-1979]Hodges,L.C., Laine,R. and Chan,S.K. Structure of the oligosaccharide chains in human alpha 1-protease inhibitor J Biol Chem (10-9-1979) 254, 8208-8212. PubMed Europe PubMed
[YEAR:25-5-1979]Johnson,D. and Travis,J. The oxidative inactivation of human alpha-1-proteinase inhibitor. Further evidence for methionine at the reactive center J Biol Chem (25-5-1979) 254, 4022-4026. PubMed Europe PubMed
[YEAR:25-6-1979]Kress,L.F., Kurecki,T., Chan,S.K. and Laskowski,M., Sr. Characterization of the inactive fragment resulting from limited proteolysis of human alpha1-proteinase inhibitor by Crotalus adamanteus proteinase II J Biol Chem (25-6-1979) 254, 5317-5320. PubMed Europe PubMed
[YEAR:28-5-1979]Matheson,N.R., Wong,P.S. and Travis,J. Enzymatic inactivation of human alpha-1-proteinase inhibitor by neutrophil myeloperoxidase Biochem Biophys Res Commun (28-5-1979) 88, 402-409. PubMed Europe PubMed
Morihara,K., Tsuzuki,H. and Oda,K. Protease and elastase of Pseudomonas aeruginosa: inactivation of human plasma alpha 1-proteinase inhibitor Infect Immun (1979) 24, 188-193. PubMed Europe PubMed PMC EPMC

1978

Bloom,J.W. and Hunter,M.J. Interactions of alpha1-antitrypsin with trypsin and chymotrypsin J Biol Chem (1978) 253, 547-559. PubMed Europe PubMed
Carp,H. and Janoff,A. Possible mechanisms of emphysema in smokers. In vitro suppression of serum elastase-inhibitory capacity by fresh cigarette smoke and its prevention by antioxidants Am Rev Respir Dis (1978) 118, 617-621. PubMed Europe PubMed
[YEAR:25-10-1978]Johnson,D. and Travis,J. Structural evidence for methionine at the reactive site of human alpha-1-proteinase inhibitor J Biol Chem (25-10-1978) 253, 7142-7144. PubMed Europe PubMed
Kress,L.F. and Paroski,E.A. Enzymatic inactivation of human serum proteinase inhibitors by snake venom proteinases Biochem Biophys Res Commun (1978) 83, 649-656. PubMed Europe PubMed

1977

[YEAR:1-6-1977]Johnson,D. and Travis,J. Inactivation of human alpha 1-proteinase inhibitor by thiol proteinases Biochem J (1-6-1977) 163, 639-641. PubMed Europe PubMed PMC EPMC

1976

[YEAR:26-11-1976]Owen,M.C., Carrell,R.W. and Brennan,S.O. The abnormality of the S variant of human alpha-1-antitrypsin Biochim Biophys Acta (26-11-1976) 453, 257-261. PubMed Europe PubMed
Pierce,J.A., Jeppsson,J.O. and Laurell,C.B. alpha-1 Antitrypsin phenotypes determined by isoelectric focusing of the cysteine-antitrypsin mixed disulfide in serum Anal Biochem (1976) 74, 227-241. PubMed Europe PubMed
Yoshida,A., Lieberman,J., Gaidulis,L. and Ewing,C. Molecular abnormality of human alpha1-antitrypsin variant (Pi-ZZ) associated with plasma activity deficiency Proc Natl Acad Sci U S A (1976) 73, 1324-1328. PubMed Europe PubMed PMC EPMC

1974

Johnson,D.A., Pannell,R.N. and Travis,J. The molecular stoichiometry of trypsin inhibition by human alpha-1-proteinase inhibitor Biochem Biophys Res Commun (1974) 57, 584-589. PubMed Europe PubMed
[YEAR:7-7-1974]Moroi,M. and Yamasaki,M. Mechanism of interaction of bovine trypsin with human alpha1-antitrypsin Biochim Biophys Acta (7-7-1974) 359, 130-141. PubMed Europe PubMed
Pannell,R., Johnson,D. and Travis,J. Isolation and properties of human plasma alpha-1-proteinase inhibitor Biochemistry (1974) 13, 5439-5445. PubMed Europe PubMed
Sandhaus,R.A. and Janoff,A. Degradation of human alpha1 antitrypsin by hepatocyte acid cathepsins. Possible role in clinical alpha1 antitrypsin deficiency Am Rev Respir Dis (1974) 110, 263-272. PubMed Europe PubMed

1973

Kueppers,F. Alpha1-antitrypsin Am J Hum Genet (1973) 25, 677-686. PubMed Europe PubMed PMC EPMC

1972

Janoff,A. Inhibition of human granulocyte elastase by serum alpha-1-antitrypsin Am Rev Respir Dis (1972) 105, 121-122. PubMed Europe PubMed
[YEAR:7-1-1972]Lieberman,J., Mittman,C. and Gordon,H.W. Alpha 1 antitrypsin in the livers of patients with emphysema Science (7-1-1972) 175, 63-65. PubMed Europe PubMed

1969

Sharp,H.L., Bridges,R.A., Krivit,W. and Freier,E.F. Cirrhosis associated with alpha-1-antitrypsin deficiency: a previously unrecognized inherited disorder J Lab Clin Med (1969) 73, 934-939. PubMed Europe PubMed M

1966

Briscoe,W.A., Kueppers,F., Davis,A.L. and Bearn,A.G. A case of inherited deficiency of serum alpha-antitrypsin associated with pulmonary emphysema Am Rev Respir Dis (1966) 94, 529-539. PubMed Europe PubMed M

1965

Laurell,C.B. Genetic variants of alpha1-antitrypsin Vox Sang (1965) 10, 363-364. PubMed Europe PubMed

1964

[YEAR:25-12-1964]Kueppers,F., Briscoe,W.A. and Bearn,A.G. Hereditary deficiency of serum alpha1-antitrypsin Science (25-12-1964) 146, 1678-1679. PubMed Europe PubMed

1962

[YEAR:15-4-1962]Schultze,H.E., Heide,K. and Haupt,H. [alpha1-Antitrypsin from human serum] Klin Wochenschr (15-4-1962) 40, 427-429. PubMed Europe PubMed

1953

Peanasky,R.J. and Laskowski,M. Partial purification of the trypsin inhibitor from blood plasma J Biol Chem (1953) 204, 153-157. PubMed Europe PubMed