JCI - Citations to alpha 1-Antitrypsin: molecular pathology, leukocytes, and tissue damage.

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alpha 1-Antitrypsin: molecular pathology, leukocytes, and tissue damage.

Published December 1, 1986
Citation Information: J Clin Invest. 1986;78(6):1427-1431. https://doi.org/10.1172/JCI112731.

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Citations to this article (221)

Title and authors	Publication	Year
Association of Serum Proteases and Acute Phase Factors Levels with Survival Outcomes in Patients with Colorectal Cancer Sebzda T, Karwacki J, Cichoń A, Modrzejewska K, Heimrath J, Łątka M, Gnus J, Gburek J	Cancers	2024
Immunological and homeostatic pathways of alpha -1 antitrypsin: a new therapeutic potential Mazzuca C, Vitiello L, Travaglini S, Maurizi F, Finamore P, Santangelo S, Rigon A, Vadacca M, Angeletti S, Scarlata S	Frontiers in Immunology	2024
Alpha-1-antitrypsin as novel substrate for S. aureus’ Spl proteases – implications for virulence Scherr F, Darisipudi MN, Börner FR, Austermeier S, Hoffmann F, Eberhardt M, Abdurrahman G, Saade C, von Eggeling F, Kasper L, Holtfreter S, Bröker BM, Kiehntopf M	Frontiers in Immunology	2024
Safety of Intravenous Administration of an AAV8 Vector Coding for an Oxidation-Resistant Human α1-Antitrypsin for the Treatment of α1-Antitrypsin Deficiency Rosenberg JB, De BP, Greco A, Gorman N, Kooner V, Chen A, Yost-Bido M, Munoz-Zuluaga C, Kaminsky SM, Rostami M, Monette S, Crystal RG, Sondhi D	Human Gene Therapy	2023
Ceruletide and Alpha-1 Antitrypsin as a Novel Combination Therapy for Ischemic Stroke. Simats A, Ramiro L, Valls R, de Ramón H, García-Rodríguez P, Orset C, Artigas L, Sardon T, Rosell A, Montaner J	Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics	2022
The Serpin Superfamily and Their Role in the Regulation and Dysfunction of Serine Protease Activity in COPD and Other Chronic Lung Diseases GA Kelly-Robinson, JA Reihill, FT Lundy, LP McGarvey, JC Lockhart, GJ Litherland, KD Thornbury, SL Martin	International journal of molecular sciences	2021
Gene therapy for alpha 1-antitrypsin deficiency with an oxidant resistant human alpha 1-antitrypsin Meredith L. Sosulski, Katie M. Stiles, Esther Z. Frenk, Fiona M. Hart, Yuki Matsumura, Bishnu P. De, Stephen M. Kaminsky, Ronald G. Crystal	JCI Insight	2020
Post-Translational Modifications of Circulating Alpha-1-Antitrypsin Protein U Lechowicz, S Rudzinski, A Jezela-Stanek, S Janciauskiene, J Chorostowska-Wynimko	International journal of molecular sciences	2020
The effect of water/ethanol solvent mixtures on interactions of an antibody selective for wild-type alpha-1-antitrypsin in complex with its antigen M Yoosefian, E Mirhaji, MZ Moghani, Y Ebrahimpour, M Fouladi	Journal of Molecular Liquids	2020
Acute Phase Proteins: Molecular Biology, Biochemistry, and Clinical Applications A Mackiewicz, I Kushner, H Baumann, A Mackiewicz, I Kushner, H Baumann		2020
Achievement of Tolerance Induction to Prevent Acute Graft-vs.-Host Disease G Thangavelu, BR Blazar	Frontiers in immunology	2019
Detection of Alpha-1 antitrypsin and Interleukin-8 in meningitis patients SK Abbas, MN Iqbal, NG Majeed		2019
Inhibitory serpins. New insights into their folding, polymerization, regulation and clearance PG Gettins, ST Olson	Biochemical Journal	2016
OxInflammation in Rett syndrome A Pecorelli, C Cervellati, J Hayek, G Valacchi	The International Journal of Biochemistry & Cell Biology	2016
Identification of biomarkers for periodontal disease using the immunoproteomics approach JP Kerishnan, S Mohammad, MS Alias, AK Mu, RD Vaithilingam, NA Baharuddin, SH Safii, ZA Rahman, YN Chen, Y Chen	PeerJ	2016
A panel of glycoproteins as candidate biomarkers for early diagnosis and treatment evaluation of B-cell acute lymphoblastic leukemia MS Cavalcante, JC Torres-Romero, MD Lobo, FB Moreno, LP Bezerra, DS Lima, JC Matos, RA Moreira, AC de Monteiro-Moreira	Biomarker Research	2016
Proteomic analysis of 4-hydroxynonenal and nitrotyrosine modified proteins in RTT fibroblasts A Pecorelli, C Cervellati, A Cortelazzo, F Cervellati, C Sticozzi, C Mirasole, R Guerranti, A Trentini, L Zolla, V Savelli, J Hayek, G Valacchi	The International Journal of Biochemistry & Cell Biology	2016
Low-density lipoprotein receptor-related protein 1 mediates 1-antitrypsin internalization in CD4+ T lymphocytes X Zhou, Z Liu, L Shapiro, J Yang, GF Burton	Journal of leukocyte biology	2015
T Helper Subsets, Peripheral Plasticity, and the Acute Phase Protein, α 1-Antitrypsin BM Baranovski, GS Freixo-Lima, EC Lewis, P Rider	BioMed Research International	2015
α-1 antitrypsin deficiency: current and future treatment options OJ McElvaney, AM Bella, NG McElvaney	Expert Opinion on Orphan Drugs	2015
Ferritin levels predict severe dengue R Soundravally, B Agieshkumar, M Daisy, J Sherin, CC Cleetus	Infection	2014
The effect of fibrin(ogen) on thrombin generation and decay: RM Kremers, RJ Wagenvoord, HC Hemker	Thrombosis and Haemostasis	2014
Subclinical Inflammatory Status in Rett Syndrome A Cortelazzo, CD Felice, R Guerranti, C Signorini, S Leoncini, A Pecorelli, G Zollo, C Landi, G Valacchi, L Ciccoli, L Bini, J Hayek	Mediators of Inflammation	2014
Emery and Rimoin's Principles and Practice of Medical Genetics DC Wallace, MT Lott, V Procaccio	Emery and Rimoin's Principles and Practice of Medical Genetics	2013
A plasma proteomic approach in Rett syndrome: classical versus preserved speech variant A Cortelazzo, R Guerranti, CD Felice, C Signorini, S Leoncini, A Pecorelli, C Landi, L Bini, B Montomoli, C Sticozzi, L Ciccoli, G Valacchi, J Hayek	Mediators of Inflammation	2013
The parallel lives of alpha1-antitrypsin deficiency and pulmonary alveolar proteinosis BC Trapnell, M Luisetti	Orphanet Journal of Rare Diseases	2013
Alpha-1 Antitrypsin Blood Levels as Indicator for the Efficacy of Cancer Treatment El-Akawi	World Journal of Oncology	2013
Varicella-zoster virus (VZV) and alpha 1 antitrypsin: a fatal outcome in a patient affected by endemic pemphigus foliaceus: Endemic pemphigus and chicken pox AM Velez, BR Smoller, W Gao, HE Grossniklaus, Z Jiao, LF Arias, SC Dudley, MS Howard	International Journal of Dermatology	2012
Production of Plasma Proteins for Therapeutic Use: Bertolini/Plasma Proteins S Chtourou, M Poulle	Production of Plasma Proteins for Therapeutic Use: Bertolini/Plasma Proteins	2012
LG3 fragment of endorepellin is a possible biomarker of severity in IgA nephropathy B Surin, E Sachon, JP Rougier, C Steverlynck, C Garreau, B Lelongt, P Ronco, R Piedagnel	PROTEOMICS	2012
Alpha-1-antitrypsin monotherapy reduces graft-versus-host disease after experimental allogeneic bone marrow transplantation I Tawara, Y Sun, EC Lewis, T Toubai, R Evers, E Nieves, T Azam, CA Dinarello, P Reddy	Proceedings of the National Academy of Sciences	2011
Alpha-1 Antitrypsin is Markedly Decreased Following Pulmonary F. tularensis Challenge JP Chambers, JJ Yu, M Jupelli, ST Weintraub, JL Lopez-Ribot, JJ Valdes, BP Arulanandam	Frontiers in Cellular and Infection Microbiology	2011
Hipertransaminazemia w przebiegu niedoboru α1-antytrypsyny u 14-miesięcznego chłopca B Jakubowska-Zając, M Czerwionka-Szaflarska	Pediatria Polska	2011
Matrix Elastin: A Promising Biomarker for Chronic Obstructive Pulmonary Disease GM Turino, S Ma, YY Lin, JO Cantor, M Luisetti	American journal of respiratory and critical care medicine	2011
Granzyme B as a novel factor involved in cardiovascular diseases Y Saito, H Kondo, Y Hojo	Journal of Cardiology	2011
Evidence for unfolded protein response activation in monocytes from individuals with alpha-1 antitrypsin deficiency TP Carroll, CM Greene, CA O'Connor, AM Nolan, SJ O'Neill, NG McElvaney	Journal of immunology (Baltimore, Md. : 1950)	2010
Proteomic analysis of peritoneal fluid of patients treated by peritoneal dialysis: effect of glucose concentration M Cuccurullo, C Evangelista, A Vilasi, M Simeoni, F Avella, E Riccio, B Memoli, A Malorni, G Capasso	Nephrology Dialysis Transplantation	2010
Protease inhibitor SERPINA1 expression in epithelial ovarian cancer K Normandin, B Péant, CL Page, M de Ladurantaye, V Ouellet, PN Tonin, DM Provencher, AM Mes-Masson	Clinical & Experimental Metastasis	2010
Chlorhexidine prevents hypochlorous acid-induced inactivation of α1-antitrypsin F Montecucco, M Bertolotto, L Ottonello, A Pende, P Dapino, A Quercioli, F Mach, F Dallegri	Clinical and Experimental Pharmacology and Physiology	2009
Alpha-1-antitrypsin is an endogenous inhibitor of proinflammatory cytokine production in whole blood GB Pott, ED Chan, CA Dinarello, L Shapiro	Journal of leukocyte biology	2009
Gene targeted therapeutics for liver disease in alpha-1 antitrypsin deficiency C McLean, CM Greene, NG McElvaney	Biologics : targets & therapy	2009
Low Serum Alpha-1 Antitrypsin (AAT) in Family Members of Individuals with Autism Correlates with PiMZ Genotype AJ Russo, L Neville, C Wroge	Biomarker insights	2009
Isoelectrofocusing and PCR Amplification-Reverse Hybridization Assay in Evaluation of Alpha-1-Antitrypsin Deficiency A Beletić, V Đorđević, A Dudvarski-Ilić, I Obradović, D Mirković, M Ilić, D Radojković, N Majkić-Singh	Journal of Medical Biochemistry	2009
Enhancement of ovalbumin-induced pulmonary eosinophilia by intranasal administration of α1-proteinase inhibitor type 2 antisense oligonucleotides HN Sung, CH Jeon, BC Gill, HR Kim, SW Cheong, JH Park	Immunology Letters	2009
Conformational Pathology of the Serpins: Themes, Variations, and Therapeutic Strategies B Gooptu, DA Lomas	Annual Review of Biochemistry	2009
Low Serum Alpha-Antitrypsin Associated with Anti-PR-3 ANCA in Autistic Children with GI Disease A J Russo, A Krigsman, B Jepson, Andrew Wakefield	Genomics insights	2009
α-1-Antitrypsin is an endogenous inhibitor of proinflammatory cytokine production in whole blood GB Pott, ED Chan, CA Dinarello, L Shapiro	Journal of leukocyte biology	2009
Mechanism of α-1 antitrypsin endocytosis by lung endothelium S Sohrab, DN Petrusca, AD Lockett, KS Schweitzer, NI Rush, Y Gu, K Kamocki, J Garrison, I Petrache	The FASEB Journal	2009
Low serum Alpha-1 Antitrypsin Associated with Anti-PR-3 AncA in Autistic Children with GI Disease AJ Russo, A Krigsman, B Jepson, A Wakefield	Genomics insights	2009
Intracellular and extracellular serpins modulate lung disease DJ Askew, GA Silverman	Journal of Perinatology	2008
Leukocyte cell surface proteinases: regulation of expression, functions, and mechanisms of surface localization CA Owen	The International Journal of Biochemistry & Cell Biology	2008
Proteomic profiling of urine identifies specific fragments of SERPINA1 and albumin as biomarkers of preeclampsia IA Buhimschi, G Zhao, EF Funai, N Harris, IE Sasson, IM Bernstein, GR Saade, CS Buhimschi	American Journal of Obstetrics and Gynecology	2008
Alpha-1 antitrypsin deficiency: A conformational disease associated with lung and liver manifestations CM Greene, SD Miller, T Carroll, C McLean, M O’Mahony, MW Lawless, SJ O’Neill, CC Taggart, NG McElvaney	Journal of Inherited Metabolic Disease	2008
Roles for proteinases in the pathogenesis of chronic obstructive pulmonary disease CA Owen	International journal of chronic obstructive pulmonary disease	2008
The relationship between protease/anti-protease profile, angiogenesis and re-epithelialisation in acute burn wounds RH Caulfield, MP Tyler, JM Austyn, P Dziewulski, DA McGrouther	Burns	2008
Isoelectric focusing phenotyping and denaturing gradient gel electrophoresis genotyping: a comparison of two methods in detection of alpha-1-antitrypsin variants M Ljujic, A Topic, A Divac, A Nikolic, N Petrovic-Stanojevic, M Surlan, M Mitic-Milikic, D Radojkovic	Translational Research	2008
Shotgun Proteomic Analysis of Vaginal Fluid From Women in Late Pregnancy LL Klein, KR Jonscher, MJ Heerwagen, RS Gibbs, JL McManaman	Reproductive sciences (Thousand Oaks, Calif.)	2008
Proteomic analysis of alpha-1-antitrypsin in immunoglobulin A nephropathy NJ Kwak, EH Wang, IY Heo, DC Jin, JH Cha, KH Lee, CW Yang, CS Kang, YJ Choi	PROTEOMICS – CLINICAL APPLICATIONS	2007
Targeted Inflammation During Oncolytic Virus Therapy Severely Compromises Tumor Blood Flow CJ Breitbach, JM Paterson, CG Lemay, TJ Falls, A McGuire, KA Parato, DF Stojdl, M Daneshmand, K Speth, D Kirn, JA McCart, H Atkins, JC Bell	Molecular Therapy	2007
Paradoxical Neutrophil Elastase Release in Endovascular Abdominal Aortic Aneurysm Repair TE Rowlands, S Homer-Vanniasinkam	Vascular and endovascular surgery	2007
Proteomic Analysis of Peritoneal Dialysate Fluid in Patients with Different Types of Peritoneal Membranes S Sritippayawan, W Chiangjong, T Semangoen, N Aiyasanon, P Jaetanawanitch, S Sinchaikul, ST Chen, S Vasuvattakul, V Thongboonkerd	Journal of Proteome Research	2007
Alpha-1-antitrypsin replacement therapy: current status H Abusriwil, RA Stockley	Current Opinion in Pulmonary Medicine	2006
Alpha-1-antitrypsin replacement therapy: current status: H Abusriwil, RA Stockley	Current Opinion in Pulmonary Medicine	2006
Analysis of Epitopes on the Unrelated Proteins Thyrotropin Receptor and alpha1-Antitrypsin which are Recognized by A10 Monoclonal Antibody U Piotrowska, G Adler	Scandinavian Journal of Immunology	2005
Respiratory Genetics K Tantisira, J Drazen	Respiratory Genetics	2005
Alpha-1-antitrypsin deficiency associated with panniculitis treated with plasma exchange therapy P Oliveira, L Paz-Melgar, MD Takahashi, MM Nico, EA Rivitti, A Mendrone, NH Tatsui	International Journal of Dermatology	2004
Alpha1-antitrypsin deficiency. 2: genetic aspects of alpha(1)-antitrypsin deficiency: phenotypes and genetic modifiers of emphysema risk DL DeMeo, EK Silverman	Thorax	2004
Fecal lactoferrin for diagnosis of symptomatic patients with ileal pouch-anal anastomosis MA Parsi, B Shen, JP Achkar, FF Remzi, JR Goldblum, J Boone, D Lin, JT Connor, VW Fazio, BA Lashner	Gastroenterology	2004
Rapid inactivation of alpha-1-proteinase inhibitor by neutrophil specific leukolysin/membrane-type matrix metalloproteinase 6 J Nie, D Pei	Experimental Cell Research	2004
Oxidative stress and coenzyme Q 10 supplementation in renal transplant recipients A Długosz, J Kuźniar, E Sawicka, Z Marchewka, J Lembas-Bogaczyk, W Sajewicz, M Boratyńska	International Urology and Nephrology	2004
Cystic fibrosis F Ratjen, G Döring	The Lancet	2003
Effect of Neutrophil Activation on Proteinase Inhibitor, Myeloperoxidase and Superoxide Production in Buffaloes GR Reddy, T More, S Kumar	Journal of Applied Animal Research	2003
Lung Biology in Health and Disease N Zamel	Lung Biology in Health and Disease: Experimental Models and Therapeutic Potential	2003
Mode of Action of RNA/DNA Oligonucleotides R Metz, M DiCola, T Kurihara, A Bailey, B Frank, B Roecklein, M Blaese	Chest	2002
Coexpression and Accumulation of Ubiquitin +1 and ZZ Proteins in Livers of Children with α 1 -Antitrypsin Deficiency SS Wu, JP de Chadarevian, L McPhaul, NE Riley, FW van Leeuwen, SW French	Pediatric and Developmental Pathology	2002
Alpha-1-antitrypsin inhibits human immunodeficiency virus type 1 L Shapiro, GB Pott, AH Ralston	The FASEB Journal	2001
Inflammatory Diseases of Blood Vessels G Hansson	Inflammatory Diseases of Blood Vessels: A Dialogue Between the Artery and the Immune System	2001
Does Reduced α1-Antitrypsin Activity Explain the Link Between Cigarette Smoking and Idiopathic Pulmonary Fibrosis? ED Chan, AH Ralston, L Shapiro	Chest	2001
Diagnosis of alpha-1-antitrypsin deficiency by DNA analysis of children with liver disease AM de TOMMASO, CL ROSSI, CA ESCANHOELA, HG SERRA, CS BERTUZZO, G HESSEL	Arquivos de Gastroenterologia	2001
Thrombin inhibition by HCII in the presence of elastase-cleaved HCII and thrombin–HCII complex H Maekawa, H Sato, DM Tollefsen	Thrombosis Research	2000
Chemical chaperones mediate increased secretion of mutant alpha 1-antitrypsin (alpha 1-AT) Z: A potential pharmacological strategy for prevention of liver injury and emphysema in alpha 1-AT deficiency JA Burrows, LK Willis, DH Perlmutter	Proceedings of the National Academy of Sciences	2000
Transfection of Nasal Mucosa with a Normal alpha1-Antitrypsin Gene in alpha1-Antitrypsin-Deficient Subjects: Comparison with Protein Therapy KL Brigham, KB Lane, B Meyrick, AA Stecenko, S Strack, DR Cannon, M Caudill, AE Canonico	Human Gene Therapy	2000
Proteinase Imbalance versus Biomechanical Stress in Pulmonary Emphysema WE Stehbens	Experimental and Molecular Pathology	2000
Handbook of Oxidants and Antioxidants in Exercise RR Jenkins, J Beard	Handbook of Oxidants and Antioxidants in Exercise	2000
Nosocomial Pneumonia UL Specks	Nosocomial Pneumonia	2000
Bioactive Proteinase 3 on the Cell Surface of Human Neutrophils: Quantification, Catalytic Activity, and Susceptibility to Inhibition EJ Campbell, MA Campbell, CA Owen	Journal of immunology (Baltimore, Md. : 1950)	2000
alpha1-Antitrypsin deficiency and toxic shock: A Japanese autopsy case O Nakade, K Kasai, M Satoh, M Yamamura, H Kakiuchi, T Kaku, M Mori	Pathology International	1999
Cigarette Smoking Causes Sequestration of Polymorphonuclear Leukocytes Released from the Bone Marrow in Lung Microvessels T Terashima, ME Klut, D English, J Hards, JC Hogg, SF van Eeden	American journal of respiratory cell and molecular biology	1999
Faecal Parameters in the Assessment of Activity in Inflammatory Bowel Disease AS der H.	Scandinavian Journal of Gastroenterology	1999
Cefoperazone Prevents the Inactivation of α1-Antitrypsin by Activated Neutrophils F Dallegri, P Dapino, N Arduino, M Bertolotto, L Ottonello	Antimicrobial agents and chemotherapy	1999
ONO-5046 is a potent inhibitor of neutrophil elastase in human pleural effusion after lobectomy T Sakuma, K Takahashi, N Ohya, K Usuda, M Handa, T Abe	European Journal of Pharmacology	1998
Transfer of the Human Alpha 1 -Antitrypsin Gene into Pulmonary Macrophages In Vivo T Ferkol, F Mularo, J Hilliard, S Lodish, JC Perales, A Ziady, M Konstan	American journal of respiratory cell and molecular biology	1998
The acute-phase protein alpha 1-antitrypsin inhibits transferrin-receptor binding and proliferation of human skin fibroblasts I Graziadei, C M Kähler, C J Wiedermann, W Vogel	Biochimica et Biophysica Acta	1998
Increased risk of chronic liver failure in adults with heterozygous ?-antitrypsin deficiency IW Graziadei, JJ Joseph, RH Wiesner, TM Therneau, KP Batts, MK Porayko	Hepatology	1998
Angiotensinogen Genotype, Sodium Reduction, Weight Loss, and Prevention of Hypertension: Trials of Hypertension Prevention, Phase II SC Hunt, NR Cook, A Oberman, JA Cutler, CH Hennekens, PS Allender, WG Walker, PK Whelton, RR Williams	Hypertension	1998
Ubiquitin and the Biology of the Cell JM Peters, JR Harris, D Finley		1998
Myeloma Complicated by α 1 -Antitrypsin Deficiency AD Heys, M Lapsley, JB Day, CD Williams	Annals of Clinical Biochemistry	1998
REVIEW: α1-Antitrypsin deficiency associated liver disease D Qu, JH Teckman, DH Perlmutter	Journal of Gastroenterology and Hepatology	1997
Airway Mucus: Basic Mechanisms and Clinical Perspectives DF Rogers, MI Lethem		1997
Chemistry and Biology of Serpins FC Church, DD Cunningham, D Ginsburg, M Hoffman, SR Stone, DM Tollefsen		1997
Proceedings of the International Symposium on Natural Antioxidants Z Zhang, E Frears, D Blake, P Winyard	Proceedings of the International Symposium on Natural Antioxidants	1996
Measurement of faecal α1-antitrypsin: Methodologies and clinical application S Choudhary, PR Gibson, MC Deacon, GP Young	Journal of Gastroenterology and Hepatology	1996
Polymerase chain reaction-mediated site-directed mutagenesis detection of Z and S alpha-1-antitrypsin alleles in family members G Hammerberg, DF Keren	Journal of Clinical Laboratory Analysis	1996
The native strains in the hydrophobic core and flexible reactive loop of a serine protease inhibitor: crystal structure of an uncleaved α1-antitrypsin at 2.7 å SE Ryu, HJ Choi, KS Kwon, KN Lee, MH Yu	Structure	1996
Cellular Internalization and Degradation of Antithrombin III-Thrombin, Heparin Cofactor II-Thrombin, and -Antitrypsin-Trypsin Complexes Is Mediated by the Low Density Lipoprotein Receptor-related Protein MZ Kounnas, FC Church, WS Argraves, DK Strickland	The Journal of biological chemistry	1996
Intracellular Polymerization of the Serpin Plasminogen Activator Inhibitor Type 2 P Mikus, T Ny	The Journal of biological chemistry	1996
Degradation of a Mutant Secretory Protein, α 1 -Antitrypsin Z, in the Endoplasmic Reticulum Requires Proteasome Activity D Qu, JH Teckman, S Omura, DH Perlmutter	The Journal of biological chemistry	1996
ARDS Acute Respiratory Distress in Adults TW Evans, C Haslett		1996
Differences in distribution and synthesis of the functional opponents 245-1245-1245-1inhibitor and neutrophil elastase in eukaryotic cells E Davids, A Ogilvie	Histochemistry and Cell Biology	1995
Plasminogen Activator Inhibitor-1 in Brain Tumors R Sawaya, M Yamamoto, OJ Rämö, ML Shi, A Rayford, JS Rao	Neurosurgery	1995
Cell surface-bound elastase and cathepsin G on human neutrophils: a novel, non-oxidative mechanism by which neutrophils focus and preserve catalytic activity of serine proteinases CA Owen, MA Campbell, PL Sannes, SS Boukedes, EJ Campbell	The Journal of Cell Biology	1995
Cathepsin G can be responsible for von Willebrand factor degradation in whole blood during storage MM Jelenska, M Kopec, I Scharrer, K Breddin	Transfusion Medicine	1995
Decreased branching, increased fucosylation and changed sialylation of alpha-1-proteinase inhibitor in breast and ovarian cancer MT Goodarzi, GA Turner	Clinica Chimica Acta	1995
Plasminogen Activator Inhibitor-1 in Brain Tumors: Relation to Malignancy and Necrosis R Sawaya, M Yamamoto, OJ Rm, ML Shi, A Rayford, JS Rao	Neurosurgery	1995
The possible role of granulocyte elastase in renal damage from acute pyelonephritis M Monga, JA Roberts	Pediatric Nephrology	1995
Glycosylation of alpha-1-proteinase inhibitor and haptoglobin in ovarian cancer: evidence for two different mechanisms GA Turner, MT Goodarzi, S Thompson	Glycoconjugate Journal	1995
CLINICAL MANIFESTATIONS OF ALPHA1-ANTITRYPSIN DEFICIENCY DH Perlmutter	Gastroenterology Clinics of North America	1995
Proteinase inhibitors in animal blood with special regard to equine pulmonary disease: ?1-proteinase inhibitor and ?2-macroglobulin A Pellegrini	Comparative Haematology International	1994
Proteolytic inactivation of alpha-1-antitrypsin by human neutrophils: involvement of multiple and interlinked cell responses to phagocytosable targets L Ottonello, P Dapino, M Scirocco, F Dallegri, C Sacchetti	European Journal of Clinical Investigation	1994
Protective role of glutathione on α1proteinase inhibitor inactivation by the myeloperoxidase system. Hypothetic study for therapeutic strategy in the management of smokers' emphysema B Gressier, S Lebegue, P Gosset, C Brunet, M Luyckx, T Dine, M Cazin, JC Cazin, B Wallaert	Fundamental & Clinical Pharmacology	1994
The Contribution of Hypoxia-Reperfusion Injury to Inflammatory Synovitis: The Influence of Reactive Oxygen Intermediates on the Transcriptional Control of Inflammation DR Blake, PG Winyard, R Marok	Annals of the New York Academy of Sciences	1994
Endothelial serpins-protectors of the vasculature? KD Forsyth, V Talbot, I Beckman	Clinical & Experimental Immunology	1994
Pathogenesis of abdominal aortic aneurysm ST MacSweeney, JT Powell, RM Greenhalgh	British Journal of Surgery	1994
Endothelial serpins--protectors of the vasculature? KD Forsyth, V Talbot, I Beckman	Clinical & Experimental Immunology	1994
Proteolysis of human native and oxidised α1-proteinase inhibitor by matrulysin and stromelysin Z Zhang, PG Winyard, K Chidwick, G Murphy, M Wardell, CR W., DR Blake	Biochimica et Biophysica Acta (BBA) - General Subjects	1994
?SZ like? ?1-antitrypsin phenotypes in PI ZZ children with liver disease DB Whitehouse, JU Lovegrove, G Mieli-Vergani, AP Mowat, DA Hopkinson	Annals of Human Genetics	1994
The effect of mesna in reversing, in vitro, the protease-antiprotease imbalance: Its reaction on the MPO-H2O2 system and on human leukocyte elastase B Gressier, S Lebegue, C Brunet, T Dine, M Luyckx, M Cazin, JC Cazin	International Journal of Pharmaceutics	1994
Protective role of glutathione on α 1 proteinase inhibitor inactivation by the myeloperoxidase system. Hypothetic study for therapeutic strategy in the management of smokers' emphysema B Gressier, S Lebegue, P Gosset, C Brunet, M Luyckx, T Dine, M Cazin, JC Cazin, B Wallaert	Fundamental & Clinical Pharmacology	1994
Scientific Foundations of Biochemistry in Clinical Practice JW Dickerson	Scientific Foundations of Biochemistry in Clinical Practice	1994
Biochemical and molecular genetic studies of abdominal aortic aneurysm in an irish population D Ramsbottom, P Fitzgerald, PA Grace, O McAnena, P Burke, P Collins, A Johnson, DT Croke, D Bouchier-Hayes	European Journal of Vascular Surgery	1994
Measurement by ELISA of equine alpha-1-proteinase inhibitor in uterine flushings from mares CL Scudamore, AD Pemberton, HR Miller, AM McDonnell, SR Thomson, A Dawson, ED Watson	Research in Veterinary Science	1994
Recombinant C1 inhibitor P5/P3 variants display resistance to catalytic inactivation by stimulated neutrophils E Eldering, CC Huijbregts, JH Nuijens, AJ Verhoeven, CE Hack	Journal of Clinical Investigation	1993
Role of the neutrophil in adult respiratory distress syndrome AC Windsor, PG Mullen, AA Fowler, HJ Sugerman	British Journal of Surgery	1993
Glycan microheterogeneity of α1-antitrypsin in serum and meconium from normal and cystic fibrosis patients by crossed immunoaffinoelectrophoresis with different lectins (Con A, LCA, WGA) S Duthel, A Revol	Clinica Chimica Acta	1993
Plasminogen-activator inhibitor type 2 (PAI-2) is a spontaneously polymerising SERPIN. Biochemical characterisation of the recombinant intracellular and extracellular forms P MIKUS, T URANO, P LILJESTROM, T NY	European Journal of Biochemistry	1993
The adhesion of labelled neutrophils on synthetic vascular grafts. An experimental porcine study H Pärsson, W Jundzill, T Jonung, J Thörne, L Norgren	European Journal of Vascular Surgery	1993
In vitro complex formation between cholesterol and α1-proteinase inhibitor S Janciauskiene, S Eriksson	FEBS Letters	1993
Neutrophil chemotaxis in the horse is not mediated by a complex of equine neutrophil elastase and equine alpha-1-proteinase inhibitor CL Scudamore, A Pemberton, ED Watson, HR Miller	British Veterinary Journal	1993
Alpha1-Antitrypsin in Tropical Pulmonary Eosinophilie D Ray, KS Krishna	Chest	1993
Increased levels of plasminogen activator inhibitor-1 (PAI-1) in human brain tumors JS Rao, A Rayford, RA Morantz, BW Festoff, R Sawaya	Journal of Neuro-Oncology	1993
Nimesulide as a Downregulator of the Activity of the Neutrophil Myeloperoxidase Pathway: Focus on the Histoprotective Potential of the Drug during Inflammatory Processes L Ottonello, P Dapino, G Pastorino, G Montagnani, F Gatti, G Guidi, F Dallegri	Drugs	1993
Constitutive and modulated expression of the human alpha 1 antitrypsin gene. Different transcriptional initiation sites used in three different cell types W Hafeez, G Ciliberto, DH Perlmutter	Journal of Clinical Investigation	1992
Inhibition of neutrophil superoxide production by human plasma α1-antitrypsin N Bucurenci, DR Blake, K Chidwick, PG Winyard	FEBS Letters	1992
Interleukin-8 and polymorphoneutrophil leucocyte activation in hemolytic uremic syndrome of childhood MM Fitzpatrick, V Shah, RS Trompeter, MJ Dillon, TM Barratt	Kidney International	1992
Proteolytic Inactivation of alpha1-Proteinase Inhibitor in vivo: Detection, Characterization and Quantitation of the Main Fragment Excreted in the Urine of Leukemia Patients R Dengler, G Eger, F Lottspeich, A Plewan, A Ogilvie, B Emmerich	Biological Chemistry Hoppe-Seyler	1992
The mechanism of Z α1-antitrypsin accumulation in the liver DA Lomas, D LI-Evans, JT Finch, RW Carrell	Nature	1992
Neutrophil activation in the haemolytic uraemic syndrome: free and complexed elastase in plasma MM Fitzpatrick, V Shah, G Filler, MJ Dillon, TM Barratt	Pediatric Nephrology	1992
Hepatobiliary Diseases J Prieto, J Rodés, DA Shafritz		1992
Biochemistry of Inflammation JT Whicher, SW Evans		1992
The cellular basis for liver injury in α1-antitrypsin deficiency DH Perlmutter	Hepatology	1991
Role of granulocyte elastase in tissue injury in patients with septic shock complicated by multiple-organ failure H Tanaka, H Sugimoto, T Yoshioka, T Sugimoto	Annals of Surgery	1991
Liver disease in infancy: a 20 year perspective G Mieli-Vergani, ER Howard, AP Mowat	Gut	1991
α1-antitrypsin deficiency and liver disease: Clinical presentation, diagnosis and treatment M Hussain, G Mieli-Vergani, AP Mowat	Journal of Inherited Metabolic Disease	1991
Increased elastase-α1-antitrypsin complex in fulminant hepatic failure: Relationship to bacterial infection and activation of coagulation PG Langley, RD Hughes, N Rolando, R Williams	Clinica Chimica Acta	1991
Plasminogen activators and inhibitors in the neuromuscular system: III. The serpin protease nexin I is synthesized by muscle and localized at neuromuscular synapses BW Festoff, JS Rao, D Hanta�	Journal of Cellular Physiology	1991
PI polymorphism in Israel: Report on six Jewish population groups S Nevo, H Cleve	American Journal of Medical Genetics	1991
New Aspects of Human Polymorphonuclear Leukocytes WH Hörl, PJ Schollmeyer		1991
Effects of Nicotine on Biological Systems F Adlkofer, K Thurau		1991
Carpal tunnel syndrome: role of occupation I Delgrosso, MA Boillat	International Archives of Occupational and Environmental Health	1991
Journal of Inherited Metabolic Disease RA Harkness, RJ Pollitt, GM Addison	Journal of Inherited Metabolic Disease	1991
The SEC receptor recognizes a pentapeptide neodomain of alpha 1-antitrypsin-protease complexes G Joslin, RJ Fallon, J Bullock, SP Adams, DH Perlmutter	The Journal of biological chemistry	1991
Alpha 1-antitrypsin deficiency, emphysema, and liver disease. Genetic basis and strategies for therapy RG Crystal	Journal of Clinical Investigation	1990
Skin-derived antileucoproteases (SKALPs): characterization of two new elastase inhibitors from psoriatic epidermis J Schalkwijk, A Chang, P Janssen, G Jongh, P Mier	British Journal of Dermatology	1990
Molecular basis of alpha 1-antitrypsin deficiency and emphysema associated with the alpha 1-antitrypsin Mmineral springs allele DT Curiel, C Vogelmeier, RC Hubbard, LE Stier, RG Crystal	Molecular and cellular biology	1990
Identification of a serpin-enzyme complex receptor on human hepatoma cells and human monocytes DH Perlmutter, GI Glover, M Rivetna, CS Schasteen, RJ Fallon	Proceedings of the National Academy of Sciences	1990
Basement membrane zone as a target for human neutrophil elastase in psoriasis W Glinski, M Jarzabek-Chorzelska, M Kuligowski, M Pierozynska-Dubowska, M Glinska-Ferenz, S Jabłonska	Archives of Dermatological Research	1990
The molecular structure and pathology ofα 1-antitrypsin RW Carell	Lung	1990
Biochemical intermediates in α1-antitrypsin deficiency: Residual family resemblance for total α1-antitrypsin, oxidized α1-antitrypsin, and immunoglobulin E after adjustment for the effect of the Pi locus EK Silverman, MA Province, EJ Campbell, JA Pierce, DC Rao, NG Martin	Genetic Epidemiology	1990
Portacaval shunt in three children with alpha-1-antitrypsin deficiency and cirrhosis: 9 to 12⅓ years later TE Starzl, KA Porter, RW Busuttil, R Pichlmayr	Hepatology	1990
α1-Antitrypsin Wbethesda: Molecular basis of an unusual α1-antitrypsin deficiency variant MD Holmes, ML Brantly, GA Fells, RG Crystal	Biochemical and Biophysical Research Communications	1990
Conformational changes in intact and papain-modified alpha1-proteinase inhibitor induced by guanidinium chloride M HERVE, C GHELIS	European Journal of Biochemistry	1990
Serum alpha- 1 -proteinase inhibitor with abnormal properties in ovarian cancer S Thompson, E Wong, BM Cantwell, GA Turner	Clinica Chimica Acta	1990
Polymerase chain reaction for detection of the alpha-1-antitrypsin Z allele in chronic liver disease AM Brind, I McIntosh, DJ Brock, OF James, MF Bassendine	Journal of Hepatology	1990
Molecular genetics of chronic liver diseases AM Brind, MF Bassendine	Bailliere's Clinical Gastroenterology	1990
Crystal structure of plakalbumin, a proteolytically nicked form of ovalbumin HT Wright, HX Qian, R Huber	Journal of Molecular Biology	1990
Dictionary of Drugs J Elks, CR Ganellin		1990
Crystallographic and Modeling Methods in Molecular Design CE Bugg, SE Ealick		1990
Serine Proteases and Their Serpin Inhibitors in the Nervous System: Regulation in Development and in Degenerative and Malignant Disease BW Festoff, D Hantaï		1990
Endocytosis and degradation of alpha 1-antitrypsin-protease complexes is mediated by the serpin-enzyme complex (SEC) receptor DH Perlmutter, G Joslin, P Nelson, C Schasteen, SP Adams, RJ Fallon	The Journal of biological chemistry	1990
Interferon beta 2/interleukin 6 modulates synthesis of alpha 1-antitrypsin in human mononuclear phagocytes and in human hepatoma cells DH Perlmutter, LT May, PB Sehgal	Journal of Clinical Investigation	1989
Synthesis of stress proteins is increased in individuals with homozygous PiZZ alpha 1-antitrypsin deficiency and liver disease DH Perlmutter, MJ Schlesinger, JA Pierce, PI Punsal, AL Schwartz	Journal of Clinical Investigation	1989
The α1-antitrypsin gene and its deficiency states RG Crystal	Trends in Genetics	1989
Alpha1-antitrypsin: Structure, function and molecular biology of the gene N Kalsheker	Bioscience Reports	1989
Structure of Human Angiotensinogen Gene I Gaillard, E Clauser, P Corvol	DNA	1989
NEUTROPHIL-MEDIATED ENDOTHELIAL INJURY IN HAEMOLYTIC URAEMIC SYNDROME KD Forsyth, MM Fitzpatrick, AC Simpson, TM Barratt, RJ Levinsky	The Lancet	1989
Structure of human neutrophil elastase in complex with a peptide chloromethyl ketone inhibitor at 1.84-A resolution MA Navia, BM McKeever, JP Springer, TY Lin, HR Williams, EM Fluder, CP Dorn, K Hoogsteen	Proceedings of the National Academy of Sciences	1989
Replacement therapy in α1-antitrypsin deficiency S Eriksson	Journal of Internal Medicine	1989
Variation in alpha-1-antitrypsin phenotypes associated with penicillamine therapy DB Whitehouse, JU Lovegrove, DA Hopkinson	Clinica Chimica Acta	1989
The prevention and management of autosomal recessive conditions. Main example: alpharantitrypsin deficiency KH Gustavson	Clinical Genetics	1989
Hemorrhagic shock and encephalopathy: Clinical, pathologic, and biochemical features M Levin, JR Pincott, M Hjelm, F Taylor, J Kay, H Holzel, R Dinwiddie, DJ Matthew	The Journal of Pediatrics	1989
Ribonuclease A Cleavage Combined with the Polymerase Chain Reaction for Detection of the Z Mutation of the Alpha-1-antitrypsin Gene T Abe, H Takahashi, MD Holmes, DT Curiel, RG Crystal	American journal of respiratory cell and molecular biology	1989
Heterozygosity of α 1 -Antitrypsin: A Health Risk? RD Feld	Critical Reviews in Clinical Laboratory Sciences	1989
Neutrophils as Effectors of Host Defense and Host Damage. Physiopathology and Perspectives of Pharmacological Manipulation C Sacchetti, F Dallegri, F Patrone	International journal of immunopathology and pharmacology	1989
Genetic studies on a new deficiency gene (PI*Ztun) at the PI locus DB Whitehouse, CM Abbott, JU Lovegrove, I McIntosh, CJ McMahon, G Mieli-Vergani, AP Mowat, DA Hopkinson	Journal of medical genetics	1989
Molecular Basis of the Liver and Lung Disease Associated with the α1-Antitrypsin Deficiency Allele Mmalton DT Curiel, MD Holmes, H Okayama, ML Brantly, C Vogelmeier, WD Travis, LE Stier, WH Perks, RG Crystal	The Journal of biological chemistry	1989
Serum α1-Antitrypsin Deficiency Associated with the Common S-type (Glu264 → Val) Mutation Results from Intracellular Degradation of α1- Antitrypsin Prior to Secretion DT Curiel, A Chytil, M Courtney, RG Crystal	The Journal of biological chemistry	1989
Binding of Type 1 Plasminogen Activator Inhibitor to the Extracellular Matrix of Cultured Bovine Endothelial Cells J Mimuro, DJ Loskutoff	The Journal of biological chemistry	1989
Proteolytic inactivation of alpha-1-proteinase inhibitor by a neutrophil metalloproteinase PE Desrochers, SJ Weiss	Journal of Clinical Investigation	1988
Elastase regulates the synthesis of its inhibitor, alpha 1-proteinase inhibitor, and exaggerates the defect in homozygous PiZZ alpha 1 PI deficiency DH Perlmutter, J Travis, PI Punsal	Journal of Clinical Investigation	1988
Sucrase-isomaltase deficiency in humans. Different mutations disrupt intracellular transport, processing, and function of an intestinal brush border enzyme HY Naim, J Roth, EE Sterchi, M Lentze, P Milla, J Schmitz, HP Hauri	Journal of Clinical Investigation	1988
Enhanced Oxidative Response of Polymorphonuclear Leukocytes from Synovial Fluids of Patients with Rheumatoid Arthritis B Dularay, CJ Elson, PA Dieppe	Autoimmunity	1988
Oxidation of proteins in rat heart and lungs by polymorphonuclear leukocyte oxidants H Fliss	Molecular and Cellular Biochemistry	1988
Inactivation of Serine Proteinase Inhibitors (Serpins) in Human Plasma by Reactive Oxidants TW STIEF, N HEIMBURGER	Biological Chemistry Hoppe-Seyler	1988
The Natural History of Liver Disease in (α1-Antitrypsin Deficient Children T Sveger	Acta Paediatrica	1988
Liver disease in children with pizz α1-antitrypsin deficiency FK Ghishan, HL Greene	Hepatology	1988
In vivo catabolism of heparin cofactor II and its complex with thrombin: Evidence for a common receptor-mediated clearance pathway for three serine proteinase inhibitors CW Pratt, FC Church, SV Pizzo	Archives of Biochemistry and Biophysics	1988
Isolation and characterization of two new low-molecular-weight protein proteinase inhibitors from the granule-rich fraction of equine neutrophilic granulocytes A Pellegrini, G Hägeli, R Fellenberg	Biochimica et Biophysica Acta (BBA) - Protein Structure and Molecular Enzymology	1988
Influence of heparin fragments on the biological activities of elastase(s) and α1 proteinase inhibitor F Redini, C Lafuma, W Hornebeck, J Choay, L Robert	Biochemical Pharmacology	1988
Neutrophil-induced Erythrocyte Injury: A Potential Cause of Erythrocyte Destruction in the Anemia Associated with Inflammatory Disease DJ Weiss, JS Klausner	Veterinary pathology	1988
Oxyradical Species and Their Relationship to Pathophysiology in Pediatric Critical Care Illness JJ Zimmerman	Critical Care Clinics	1988
Phagocytes and Disease MS Klempner, B Styrt, J Ho		1988
Distinct and additive effects of elastase and endotoxin on expression of alpha 1 proteinase inhibitor in mononuclear phagocytes DH Perlmutter, PI Punsal	The Journal of biological chemistry	1988
cis-dichlorodiammineplatinum (II) as a selective modifier of the oxidation-sensitive reactive-center methionine in alpha 1-antitrypsin SL Gonias, MW Swaim, MF Massey, SV Pizzo	The Journal of biological chemistry	1988
Lipopolysaccharide modulates the expression of alpha 1 proteinase inhibitor and other serine proteinase inhibitors in human monocytes and macrophages C Barbey-Morel, JA Pierce, EJ Campbell, DH Perlmutter	Journal of Experimental Medicine	1987
SC-39026, a specific human neutrophil elastase inhibitor A Nakao, RA Partis, GP Jung, RA Mueller	Biochemical and Biophysical Research Communications	1987
Retroviral mediated transfer and expression of human alpha1-antitrypsin in cultured cells FD Ledley, HE Grenett, DP Bartos, SL Woo	Gene	1987
Characterization of the M1(Ala213) type of .alpha.1-antitrypsin, a newly recognized, common "normal" .alpha.1-antitrypsin haplotype T Nukiwa, M Brantly, F Ogushi, G Fells, K Satoh, L Stier, M Courtney, RG Crystal	Biochemistry	1987
Clonal Gene Therapy: Transplanted Mouse Fibroblast Clones Express Human α1-Antitrypsin Gene in Vivo RI Garver, A Chytil, M Courtney, RG Crystal	Science	1987
alpha 1-Antitrypsin nullGranite Falls, a nonexpressing alpha 1-antitrypsin gene associated with a frameshift to stop mutation in a coding exon T Nukiwa, H Takahashi, M Brantly, M Courtney, RG Crystal	The Journal of biological chemistry	1987
New Comprehensive Biochemistry S Yamamoto	New Comprehensive Biochemistry	1983
Liver Disorders in Childhood AP Mowat	Liver Disorders in Childhood	1979

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