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R G Crystal
Published May 1, 1990
Citation Information: J Clin Invest. 1990;85(5):1343-1352. https://doi.org/10.1172/JCI114578.
Citation Information: J Clin Invest. 1990;85(5):1343-1352. https://doi.org/10.1172/JCI114578.
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R G Crystal
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Citations to this article (398)
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PiZ Mouse Liver Accumulates Polyubiquitin Conjugates That Associate with Catalytically Active 26S Proteasomes
Christopher J. Haddock, Keith Blomenkamp, Madhav Gautam, Jared James, Joanna Mielcarska, Edward Gogol, Jeffrey Teckman, Dorota Skowyra |
PloS one | 2014 |
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Diagnosis of alpha-1 antitrypsin deficiency: Modalities, indications and diagnosis strategy
M Balduyck, MF Odou, F Zerimech, N Porchet, JJ Lafitte, B Maitre |
Revue des Maladies Respiratoires | 2014 |
|
Reference Module in Biomedical Sciences
DL Sackett, RB Haynes |
2014 | |
|
Encyclopedia of Medical Immunology: Allergic Diseases
IR Mackay, NR Rose, DK Ledford, RF Lockey |
2014 | |
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Disorders of Protein Misfolding: Alpha-1-Antitrypsin Deficiency as Prototype
GA Silverman, SC Pak, DH Perlmutter |
The Journal of Pediatrics | 2013 |
|
Déficits en alpha-1 antitrypsine
F Lebargy |
EMC - Pneumologie | 2013 |
|
Targeting intracellular degradation pathways for treatment of liver disease caused by α1-antitrypsin deficiency
Y Wang, DH Perlmutter |
Pediatric Research | 2013 |
|
Gene polymorphisms and chronic obstructive pulmonary disease
X Wu, B Yuan, E López, C Bai, X Wang |
Journal of Cellular and Molecular Medicine | 2013 |
|
The design of a new truncated and engineered alpha1-antitrypsin based on theoretical studies: an antiprotease therapeutics for pulmonary diseases
N Pirooznia, S Hasannia, SS Arab, AS Lotfi, M Ghanei, A Shali |
Theoretical Biology and Medical Modelling | 2013 |
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Intrapleural Administration of an AAVrh.10 Vector Coding for Human α1-Antitrypsin for the Treatment of α1-Antitrypsin Deficiency
MJ Chiuchiolo, SM Kaminsky, D Sondhi, NR Hackett, JB Rosenberg, EZ Frenk, Y Hwang, BG van de Graaf, JA Hutt, G Wang, J Benson, RG Crystal |
Human Gene Therapy Clinical Development | 2013 |
|
Distinguishing alpha1-antitrypsin deficiency from asthma
D Siri, H Farah, DK Hogarth |
Annals of Allergy, Asthma & Immunology | 2013 |
|
Prevalence and Risk Factors for Liver Involvement in Individuals with PiZZ-related Lung Disease
MF Dawwas, SE Davies, WJ Griffiths, DA Lomas, GJ Alexander |
American journal of respiratory and critical care medicine | 2013 |
|
A novel alpha1-antitrypsin null variant ( PiQ0 Milano )
R Rametta |
World journal of hepatology | 2013 |
|
Alpha-1 Antitrypsin: A Potent Anti-Inflammatory and Potential Novel Therapeutic Agent
DA Bergin, K Hurley, NG McElvaney, EP Reeves |
Archivum Immunologiae et Therapiae Experimentalis | 2012 |
|
Novel treatment strategies for liver disease due to α1-antitrypsin deficiency
N Maurice, DH Perlmutter |
Clinical and Translational Science | 2012 |
|
Immune-directed therapy for type 1 diabetes at the clinical level: the Immune Tolerance Network (ITN) experience
MR Ehlers, GT Nepom |
The review of diabetic studies : RDS | 2012 |
|
α1-Antitrypsin Deficiency
D Perlmutter |
Zakim and Boyer s Hepatology | 2012 |
|
Generating cells of the gastrointestinal system: current approaches and applications for the differentiation of human pluripotent stem cells
A Wang, M Sander |
Journal of Molecular Medicine | 2012 |
|
Therapeutic Drug Monitoring
A Stone, J Bornhorst |
Therapeutic Drug Monitoring | 2012 |
|
Alpha-1 antitrypsin deficiency: a commonly overlooked cause of lung disease
SK Brode, SC Ling, KR Chapman |
CMAJ : Canadian Medical Association journal = journal de l'Association medicale canadienne | 2012 |
|
Alpha-1-antitrypsin inhibits nitric oxide production
ED Chan, GB Pott, PE Silkoff, AH Ralston, CL Bryan, L Shapiro |
Journal of leukocyte biology | 2012 |
|
Spontaneous hepatic repopulation in transgenic mice expressing mutant human alpha 1-anti-trypsin by wildtype donor hepatocytes
Jianqiang Ding, Govardhana Yannam, Namita Roy-Chowdhury, Tunda Hidvegi, Hesham Basma, Stephen Rennard, Ronald Wong, Yesim Avsar, Chandan Guha, David Perlmutter, Ira Fox, Jayanta Roy-Chowdhury |
Journal of Clinical Investigation | 2011 |
|
Hepatic fibrosis and carcinogenesis in α1-antitrypsin deficiency: a prototype for chronic tissue damage in gain-of-function disorders
DH Perlmutter, GA Silverman |
Cold Spring Harbor perspectives in biology | 2011 |
|
Dilatation of the ascending aorta and serum alpha 1-antitrypsin level in patients with bicuspid aortic valve
KO Kilickesmez, O Abaci, C Kocas, A Yildiz, A Kaya, B Okcun, S Kucukoglu |
Heart and Vessels | 2011 |
|
Insights into the Pathogenesis and Treatment of Cancer from Inborn Errors of Metabolism
A Erez, OA Shchelochkov, SE Plon, F Scaglia, B Lee |
The American Journal of Human Genetics | 2011 |
|
Alpha-1-Antitrypsin Deficiency: Importance of Proteasomal and Autophagic Degradative Pathways in Disposal of Liver Disease–Associated Protein Aggregates
DH Perlmutter |
Annual Review of Medicine | 2011 |
|
The role of augmentation therapy in alpha-1 antitrypsin deficiency
F Kueppers |
Current Medical Research and Opinion | 2011 |
|
Alpha-1 Antitrypsin Deficiency
DH Perlmutter |
Schiff s Diseases of the Liver Schiff/Schiff s Diseases of the Liver | 2011 |
|
Schiff's Diseases of the Liver: Schiff/Schiff's Diseases of the Liver
H Malhi, GJ Gores |
Schiff's Diseases of the Liver: Schiff/Schiff's Diseases of the Liver | 2011 |
|
Identification of a rare p.G320R alpha-1-antitrypsin variant in emphysema and lung cancer patients
M Ljujic, A Topic, A Nikolic, A Divac, M Grujic, M Mitic-Milikic, D Radojkovic |
Genetics and molecular biology | 2010 |
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Evaluation of activity and phenotype of α1-antitrypsin in a civil population with respiratory complications following exposure to sulfur mustard 20 years ago
M Shohrati, N Shamspour, F Babaei, AA Harandi, A Mohsenifar, J Aslani, M Ghanei |
Biomarkers | 2010 |
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Inheritable Forms of Liver Disease
BR Bacon |
GI/Liver Secrets | 2010 |
|
Development of novel microarray methodology for the study of mutations in the SERPINA1 and ADRB2 genes—Their association with Obstructive Pulmonary Disease and Disseminated Bronchiectasis in Greek patients
A Papatheodorou, P Makrythanasis, M Kaliakatsos, A Dimakou, D Orfanidou, C Roussos, E Kanavakis, M Tzetis |
Clinical Biochemistry | 2010 |
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Identification of a rare p.G320R alpha-1-antitrypsin variant in emphysema and lung cancer patients
M Ljujic, A Topic, A Nikolic, A Divac, M Grujic, M Mitic-Milikic, D Radojkovic |
Genetics and molecular biology | 2010 |
|
Augmentation therapy in alpha-1 antitrypsin deficiency: advances and controversies
AR Tonelli, ML Brantly |
Therapeutic advances in respiratory disease | 2010 |
|
In silico analysis of alpha1-antitrypsin variants: the effects of a novel mutation
S Denden, N Leban, D Hayek, J Knani, JB Chibani, AH Khelil |
Genetics and molecular biology | 2010 |
|
Alpha-1 antitrypsin gene polymorphism in Chronic Obstructive Pulmonary Disease (COPD)
S Denden, AH Khelil, J Knani, R Lakhdar, P Perrin, G Lefranc, JB Chibani |
Genetics and molecular biology | 2010 |
|
Polymorphisms in the SERPINA1 (Alpha-1-Antitrypsin) Gene are Associated with Severe Chronic Rhinosinusitis Unresponsive to Medical Therapy
SJ Kilty, Y Bossé, C Cormier, LM Endam, MY Desrosiers |
American journal of rhinology & allergy | 2010 |
|
Molecular Pathology of Liver Diseases
SP Monga |
2010 | |
|
Emphysema Mediated by Lung Overexpression of ADAM10
H Saitoh, PL Leopold, BG Harvey, TP O’Connor, S Worgall, NR Hackett, RG Crystal |
Clinical and Translational Science | 2009 |
|
New Findings in PiZZ alpha1-antitrypsin deficiency-related panniculitis. Demonstration of skin polymers and high dosing requirements of intravenous augmentation therapy
B Gross, M Grebe, M Wencker, JK Stoller, LM Bjursten, S Janciauskiene |
Dermatology (Basel, Switzerland) | 2009 |
|
Hepatoid carcinoma of the skin: spontaneous rat skin hepatoid carcinoma with eosinophilic globules and crystals immunoreactive to α-1-antitrypsin
M Sutoh, M Chiba, K Kasai, T Miura, H Nozaka, K Washiya, E Okusawa, N Oyama, C Tsutaya, M Tsushima, Y Terayama, T Funyu, T Sato |
Medical Molecular Morphology | 2009 |
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The alpha-1-antitrypsin gene promoter in human A549 lung derived cells, and a novel transcription initiation site
K Morgan, S Chappell, T Guetta-Baranés, S Morley, N Kalsheker |
The International Journal of Biochemistry & Cell Biology | 2009 |
|
Screening for Alpha 1 antitrypsin deficiency in Tunisian subjects with obstructive lung disease: a feasibility report
S Denden, M Zorzetto, F Amri, J Knani, S Ottaviani, R Scabini, M Gorrini, I Ferrarotti, I Campo, JB Chibani, AH Khelil, M Luisetti |
Orphanet Journal of Rare Diseases | 2009 |
|
Copy number variation in the mouse genome: implications for the mouse as a model organism for human disease
G Cutler, PD Kassner |
Cytogenetic and Genome Research | 2009 |
|
Real time PCR detection of the PI*Z and PI*S mutations associated with alpha-1 antitrypsin deficiency
CL Bartels, AL Marchetti, WE Highsmith, GJ Tsongalis |
American journal of translational research | 2009 |
|
Control of inflammation with alpha1-antitrypsin: A potential treatment for islet transplantation and new-onset type 1 diabetes
GC Weir, M Koulamnda |
Current Diabetes Reports | 2009 |
|
Alfa-1-antitrypsin deficiency panniculitis acquired after liver transplant and successfully treated with retransplant
R Fernández-Torres, J García-Silva, O Robles, A Otero, MÁ Vázquez, E Fonseca |
Journal of the American Academy of Dermatology | 2009 |
|
Z and Mmalton-1-antitrypsin deficiency-associated hepatocellular carcinoma: a genetic study
P Francalanci, FM Santorelli, S Saccani, MF Bonetti, D Medicina, P Coni, G Faa, F Callea |
Liver International | 2009 |
|
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 |
|
Dysfunctional glycogen storage in a mouse model of alpha1-antitrypsin deficiency
RH Hubner, PL Leopold, M Kiuru, BP De, A Krause, RG Crystal |
American journal of respiratory cell and molecular biology | 2008 |
|
Metabolic disorders and storage diseases
|
2008 | |
|
Alpha-1-Antitrypsin Deficiency: A Case of a Two-year Old Boy with Inherited Disease
A Petlichkovski, S Peova, D Trajkov, T Arsov, A Strezova, S Hristomanova, E Djulejic, J Petrov, M Spiroski |
Macedonian Journal of Medical Sciences | 2008 |
|
Autophagic disposal of the aggregation-prone protein that causes liver inflammation and carcinogenesis in α-1-antitrypsin deficiency
DH Perlmutter |
Cell Death and Differentiation | 2008 |
|
Effective DNA inhibitors of cathepsin g by in vitro selection
B Gatto, E Vianini, L Lucatello, C Sissi, D Moltrasio, R Pescador, R Porta, M Palumbo |
International journal of molecular sciences | 2008 |
|
Polymorphisms in the SERPINA1 Gene and the Risk of Chronic Obstructive Pulmonary Disease in a Korean Population
SI Cha, JE Choi, JM Lee, SS Yoo, CH Kim, WK Lee, TH Jung, NS Kim, JY Park |
Tuberculosis and Respiratory Diseases | 2008 |
|
Serum activity of alanine aminotransferase (ALT) as an indicator of health and disease
WR Kim, SL Flamm, AM di Bisceglie, HC Bodenheimer |
Hepatology | 2008 |
|
Evaluation of a new Sebia isoelectrofocusing kit for α1-antitrypsin phenotyping with the Hydrasys®System
F Zerimech, G Hennache, F Bellon, G Barouh, JJ Lafitte, N Porchet, M Balduyck |
Clinical Chemistry and Laboratory Medicine | 2008 |
|
Issues in the diagnosis of α1-antitrypsin deficiency
G Rachelefsky, DK Hogarth |
Journal of Allergy and Clinical Immunology | 2008 |
|
Gene Transfer in the Lung
TR Flotte |
Mechanical Ventilation | 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 |
|
Alpha 1 antitrypsin polymorphism in the tunisian population with special reference to pulmonary disease
S Denden, AH Khelil, P Perrin, H Daimi, N Leban, A Ouaja, K Mahdouani, L Hlioui, G Lefranc, JB Chibani |
Pathologie Biologie | 2008 |
|
Genetic Influences in Common Respiratory Disorders
ML Workman, C Winkelman |
Critical Care Nursing Clinics of North America | 2008 |
|
Intravenous Human Plasma-Derived Augmentation Therapy in α 1 -Antitrypsin Deficiency: From Pharmacokinetic Analysis to Individualizing Therapy
NP Zamora, RV Pla, PG Rio, RJ Margaleff, FR Frias, JB Ronsano |
The Annals of pharmacotherapy | 2008 |
|
Campylobacter
I Nachamkin, CM Szymanski, MJ Blaser |
Campylobacter | 2008 |
|
Modification of Gene Expression and Increase inα1-Antitrypsin (α1-AT) Secretion After Homologous Recombination inα1-AT-Deficient Monocytes
GL McNab, A Ahmad, D Mistry, RA Stockley |
Human Gene Therapy | 2007 |
|
In vivo post-transcriptional gene silencing of α-1 antitrypsin by adeno-associated virus vectors expressing siRNA
PE Cruz, C Mueller, TL Cossette, A Golant, Q Tang, SG Beattie, M Brantly, M Campbell-Thompson, KS Blomenkamp, JH Teckman, TR Flotte |
Laboratory Investigation | 2007 |
|
A polymorphism of the alpha1-antitrypsin gene represents a risk factor for liver disease
S Chappell, N Hadzic, R Stockley, T Guetta-Baranes, K Morgan, N Kalsheker |
Hepatology | 2007 |
|
Genotypes and serum concentrations of human alpha-1-antitrypsin "P" protein variants in a clinical population
JA Bornhorst, FR Calderon, M Procter, W Tang, ER Ashwood, R Mao |
Journal of clinical pathology | 2007 |
|
GERMAN COCKROACH FRASS PROTEASES CLEAVE PRO-MATRIX METALLOPROTEINASE-9
VS Hughes, K Page |
Experimental Lung Research | 2007 |
|
Pädiatrie
MJ Lentze, FJ Schulte, J Schaub, J Spranger |
Pädiatrie | 2007 |
|
The effect of total starvation and very low energy diet in lean men on kinetics of whole body protein and five hepatic secretory proteins
PR Afolabi, F Jahoor, AA Jackson, J Stubbs, AM Johnstone, P Faber, G Lobley, E Gibney, M Elia |
American journal of physiology. Endocrinology and metabolism | 2007 |
|
Mutant fibrinogen cleared from the endoplasmic reticulum via endoplasmic reticulum-associated protein degradation and autophagy: an explanation for liver disease
KB Kruse, A Dear, ER Kaltenbrun, BE Crum, PM George, SO Brennan, AA McCracken |
The American Journal of Pathology | 2006 |
|
Pathogenesis of Chronic Liver Injury and Hepatocellular Carcinoma in Alpha-1-Antitrypsin Deficiency:
DH Perlmutter |
Pediatric Research | 2006 |
|
Characterization of an ERAD Gene as VPS30/ATG6 Reveals Two Alternative and Functionally Distinct Protein Quality Control Pathways: One for Soluble Z Variant of Human α-1 Proteinase Inhibitor (A1PiZ) and Another for Aggregates of A1PiZ
KB Kruse, JL Brodsky, AA McCracken |
Molecular biology of the cell | 2006 |
|
Genetic medicines: treatment strategies for hereditary disorders
TP O'Connor, RG Crystal |
Nature Reviews Genetics | 2006 |
|
Recombinant human alpha-1 proteinase inhibitor: towards therapeutic use
E Karnaukhova, Y Ophir, B Golding |
Amino Acids | 2006 |
|
Effect of Metabolite I of Erdosteine on the Release of Human Neutrophil Elastase
PC Braga, MD Sasso, M Culici, P Verducci, RL Verso, L Marabini |
Pharmacology | 2006 |
|
Screening of alpha-1-antitrypsin gene by denaturing gradient gel electrophoresis (DGGE)
M Ljujic, A Nikolic, A Divac, V Djordjevic, D Radojkovic |
Journal of Biochemical and Biophysical Methods | 2006 |
|
Multi-Center Study: The Biochemical Efficacy, Safety and Tolerability of a New α 1 -Proteinase Inhibitor, Zemaira
JM Stocks, M Brantly, D Pollock, A Barker, F Kueppers, C Strange, JF Donohue, R Sandhaus |
COPD | 2006 |
|
High Levels of Persistent Expression of α1-Antitrypsin Mediated by the Nonhuman Primate Serotype rh.10 Adeno-associated Virus Despite Preexisting Immunity to Common Human Adeno-associated Viruses
BP De, A Heguy, NR Hackett, B Ferris, PL Leopold, J Lee, L Pierre, G Gao, JM Wilson, RG Crystal |
Molecular Therapy | 2006 |
|
C-Terminal Amino Acid Alteration rather than Late Termination Causes Complete Deficiency of Thyroxine-Binding Globulin CD-NeuIsenburg
LC Moeller, A Fingerhut, H Lahner, H Grasberger, B Weimer, J Happ, K Mann, OE Janssen |
The Journal of clinical endocrinology and metabolism | 2006 |
|
YopM of Yersinia enterocolitica specifically interacts with α1-antitrypsin without affecting the anti-protease activity
G Heusipp, K Spekker, S Brast, S Fälker, MA Schmidt |
Microbiology | 2006 |
|
Genome–environment interactions in the molecular pathogenesis of dilated cardiomyopathy
W Poller, U Kühl, C Tschoepe, M Pauschinger, H Fechner, HP Schultheiss |
Journal of Molecular Medicine | 2005 |
|
Genetics of intracranial aneurysms
YM Ruigrok, GJ Rinkel, C Wijmenga |
The Lancet Neurology | 2005 |
|
alpha1-Antitrypsin deficiency-associated panniculitis: case report and review of treatment options
PG Ortiz, BG Skov, E Benfeldt |
Journal of the European Academy of Dermatology and Venereology | 2005 |
|
Acute phase mediator oncostatin M regulates affinity of α1-protease inhibitor for concanavalin A in hepatoma-derived but not lung-derived epithelial cells
P Kulig, J Cichy |
Cytokine | 2005 |
|
Laboratory Techniques in Biochemistry and Molecular Biology
S Chen, KM Madsen, CC Tisher, A Agarwal |
Laboratory Techniques in Biochemistry and Molecular Biology | 2005 |
|
Molecular aspects of biochemical markers
I Yuasa, K Umetsu |
Legal Medicine | 2005 |
|
Comparison of the properties of rare variants of α1-proteinase inhibitor expressed in COS-1 cells and assessment of their potential as risk factors in human disease
S Ray, TD Mickleborough, JL Brown |
Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease | 2005 |
|
Epidemiology and carcinogenesis of hepatocellular carcinoma
TY Leong, AS Leong |
HPB : the official journal of the International Hepato Pancreato Biliary Association | 2005 |
|
Aralast: A New α 1 -Protease Inhibitor for Treatment of α-Antitrypsin Deficiency
SG Louie, DA Sclar, MA Gill |
The Annals of pharmacotherapy | 2005 |
|
α 1 -Antitrypsin Binds to and Interferes with Functionality of EspB from Atypical and Typical Enteropathogenic Escherichia coli Strains
S Knappstein, T Ide, MA Schmidt, G Heusipp |
Infection and immunity | 2004 |
|
Intrapleural administration of a serotype 5 adeno-associated virus coding for α1-antitrypsin mediates persistent, high lung and serum levels of α1-antitrypsin
B De, A Heguy, PL Leopold, N Wasif, RJ Korst, NR Hackett, RG Crystal |
Molecular Therapy | 2004 |
|
Alpha-1-antitrypsin deficiency: diagnosis and treatment
DH Perlmutter |
Clinics in Liver Disease | 2004 |
|
Concentration of hydroxyproline in blood: A biological marker in occupational exposure to asbestos and its relationship with Pi*Z and Pi*S polymorphism in the alpha-1 antitrypsin gene
S Mas, X Casterad, N Laso, MJ Lafuente, R Panades, A Calleja, S Hernandez, D Turuguet, R Deulofeu, A Ballesta, C Ascaso, A Lafuente |
American Journal of Industrial Medicine | 2004 |
|
Alpha-1 antitrypsin deficiency: A study from the registry
A Khoor |
Human Pathology | 2004 |
|
The bronchopulmonary pathology of alpha-1 antitrypsin (AAT) deficiency: Findings of the death review committee of the national registry for individuals with severe deficiency of alpha-1 antitrypsin
JF Tomashefski, RG Crystal, HP Wiedemann, E Mascha, JK Stoller |
Human Pathology | 2004 |
|
Lack of Effect of Oral 4-Phenylbutyrate on Serum Alpha-1-Antitrypsin in Patients with α-1-Antitrypsin Deficiency: A Preliminary Study:
JH Teckman |
Journal of Pediatric Gastroenterology and Nutrition | 2004 |
|
Endoplasmic Reticulum (ER) Stress Induced by a Neurovirulent Mouse Retrovirus Is Associated with Prolonged BiP Binding and Retention of a Viral Protein in the ER
DE Dimcheff, MA Faasse, FJ McAtee, JL Portis |
The Journal of biological chemistry | 2004 |
|
Encyclopedia of Gastroenterology
RA Liddle |
Encyclopedia of Gastroenterology | 2003 |
|
A physiological and biochemical model for digestion in the ectoparasitic mite, Psoroptes ovis (Acari: Psoroptidae)
KA Hamilton, AJ Nisbet, MJ Lehane, MA Taylor, PF Billingsley |
International Journal for Parasitology | 2003 |
|
Airway hyperresponsiveness in a large group of subjects with alpha1-antitrypsin deficiency: a cross-sectional controlled study
M Malerba, A Radaeli, L Ceriani, C Tantucci, V Grassi |
Journal of Internal Medicine | 2003 |
|
Effects of a caffeine-free Cola nitida nuts extract on elastase/alpha-1-proteinase inhibitor balance
DA Daels-Rakotoarison, G Kouakou, B Gressier, T Dine, C Brunet, M Luyckx, F Bailleul, F Trotin |
Journal of Ethnopharmacology | 2003 |
|
Cost-Effectiveness Analysis of Augmentation Therapy for Severe α 1 -Antitrypsin Deficiency
TR Gildea, KM Shermock, ME Singer, JK Stoller |
American journal of respiratory and critical care medicine | 2003 |
|
Alpha-1-antitrypsin expression in the lung is increased by airway delivery of gene-transfected macrophages
D Zhang, M Wu, DE Nelson, R Pasula, WJ Martin |
Gene Therapy | 2003 |
|
Genetic diversity of the alpha-1-antitrypsin gene in Africans identified using a novel genotyping assay
VM Hayes |
Human Mutation | 2003 |
|
Liver injury in alpha-1 antitrypsin deficiency
David Perlmutter |
Journal of Clinical Investigation | 2002 |
|
Pi*S and Pi*Z alpha 1 antitrypsin polymorphism and the risk for asbestosis in occupational exposure to asbestos
MJ Lafuente, X Casterad, N Laso, S Mas, R Panades, A Calleja, S Hernandez, D Turuguet, A Ballesta, C Ascaso, A Lafuente |
Toxicology Letters | 2002 |
|
α1-Antitrypsin null alleles: evidence for the recurrence of the L353fsX376 mutation and a novel G→A transition in position +1 of intron IC affecting normal mRNA splicing
S Seixas, C Mendonça, F Costa, J Rocha |
Clinical Genetics | 2002 |
|
Oncostatin M induced alpha1-antitrypsin (AAT) gene expression in Hep G2 cells is mediated by a 3' enhancer
K Morgan, P Marsters, S Morley, D Gent, A Hejazi, M Backx, ER Thorpe, N Kalsheker |
Biochemical Journal | 2002 |
|
Genetics of obstructive airways disease
E Halapi, H Hakonarson |
Immunology and Allergy Clinics of North America | 2002 |
|
Combined liver-kidney transplantation in a 15-year-old boy with alpha1-antitrypsin deficiency
M Loreno, P Boccagni, P Rigotti, R Naccarato, P Burra |
Journal of Hepatology | 2002 |
|
AGA technical review on the evaluation of liver chemistry tests
RM Green, S Flamm |
Gastroenterology | 2002 |
|
Rapid Cycle Real-Time PCR — Methods and Applications
W Dietmaier, C Wittwer, N Sivasubramanian |
2002 | |
|
Retinoic acid attenuates cytokine-driven fibroblast degradation of extracellular matrix in three-dimensional culture
YK Zhu, X Liu, RF Ertl, T Kohyama, FQ Wen, H Wang, JR Spurzem, DJ Romberger, SI Rennard |
American journal of respiratory cell and molecular biology | 2001 |
|
Alpha-1-antitrypsin inhibits human immunodeficiency virus type 1
L Shapiro, GB Pott, AH Ralston |
The FASEB Journal | 2001 |
|
Patty's Toxicology
DL Hunt, JJ Tulis |
Patty's Toxicology | 2001 |
|
Collaborative interactions between neutrophil elastase and metalloproteinases in extracellular matrix degradation in three-dimensional collagen gels
Y Zhu, X Liu, CM Sköld, H Wang, T Kohyama, FQ Wen, RF Ertl, SI Rennard |
Respiratory Research | 2001 |
|
Synthesis and evaluation of novel β-lactam inhibitors of human leukocyte elastase
KP Koteva, AM Cantin, WA Neugebauer, E Escher |
Canadian Journal of Chemistry | 2001 |
|
Rare alpha-1-antitrypsin phenotypes and liver-test abnormalities during infancy
K Pittschieler |
Acta Paediatrica | 2001 |
|
The Folding of Alpha-1-Proteinase Inhibitor: Kinetic vs Equilibrium Control
ST Tran, A Shrake |
Archives of Biochemistry and Biophysics | 2001 |
|
Inflammatory Diseases of Blood Vessels: Pathogenesis
P Cockwell, C Savage |
Inflammatory Diseases of Blood Vessels: A Dialogue Between the Artery and the Immune System | 2001 |
|
α1-Antitrypsin Deficiency: Biological Answers to Clinical Questions
RJ Coakley, C Taggart, S ONeill, NG McElvaney |
The American Journal of the Medical Sciences | 2001 |
|
Rapid Cycle Real-Time PCR
S Meuer, C Wittwer, KI Nakagawara |
2001 | |
|
Heterozygous M3Mmalton α1-Antitrypsin Deficiency Associated with End-Stage Liver Disease: Case Report and Review
V Canva, S Piotte, JP Aubert, N Porchet, M Lecomte-Houcke, G Huet, T Zenjari, D Roumilhac, FR Pruvot, P Degand, JC Paris, M Balduyck |
Clinical chemistry | 2001 |
|
Sequential processing of human ProIL-1beta by caspase-1 and subsequent folding determined by a combined in vitro and in silico approach
P W Swaan, D L Knoell, F Helsper, M D Wewers |
Pharmaceutical Research | 2001 |
|
Alphain1-antitrypsin deficiency
DH Perlmutter |
Current Treatment Options in Gastroenterology | 2000 |
|
α1-Antitrypsin in serum determined by capillary isoelectric focusing
A Lupi, S Viglio, M Luisetti, M Gorrini, P Coni, G Faa, G Cetta, P Iadarola |
Electrophoresis | 2000 |
|
Mouse models of chronic lung infection withPseudomonas Aeruginosa: Models for the study of cystic fibrosis
PK Stotland, D Radzioch, MM Stevenson |
Pediatric Pulmonology | 2000 |
|
Alpha-1-antitrypsin stimulates fibroblast proliferation and procollagen production and activates classical MAP kinase signalling pathways
K Dabbagh, GJ Laurent, A Shock, P Leoni, J Papakrivopoulou, RC Chambers |
Journal of Cellular Physiology | 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 |
|
Differential regulation of gene activity and chromatin structure within the human serpin gene cluster at 14q32.1 in macrophage microcell hybrids
P Rollini, RE Fournier |
Nucleic Acids Research | 2000 |
|
Utilization of the 1,2,5-thiadiazolidin-3-one 1,1 dioxide scaffold in the design of potent inhibitors of serine proteases: SAR studies using carboxylates
R Kuang, JB Epp, S Ruan, LS Chong, R Venkataraman, J Tu, S He, TM Truong, WC Groutas |
Bioorganic & Medicinal Chemistry | 2000 |
|
Functional effects of the inhibition of the cysteine protease activity of the major house dust mite allergen Der p 1 by a novel peptide-based inhibitor
John, Rusznak, Ramjee, Lamont, Abrahamson, Hewitt |
Clinical & Experimental Allergy | 2000 |
|
Genotyping of α-antitrypsin in ten Croatian families
I Žuntar, E Topić, Z Jurčić, A Zubčić |
Clinical Biochemistry | 2000 |
|
LIVER INJURY IN ALPHA1-ANTITRYPSIN DEFICIENCY
DH Perlmutter |
Clinics in Liver Disease | 2000 |
|
Nosocomial Pneumonia
UL Specks |
Nosocomial Pneumonia | 2000 |
|
Lung Elastin and Matrix
BC Starcher |
Chest | 2000 |
|
Alpha1-Antitrypsin Deficiency May Be a Risk Factor for Duodenal Ulcer in Patients with Helicobacter pylori Infection
AN Floré |
Scandinavian Journal of Gastroenterology | 2000 |
|
Klinische Gastroenterologie und Stoffwechsel
G Adler, C Beglinger, MP Manns, S Müller-Lissner, W Schmiegel |
2000 | |
|
Pathologie der Leber und Gallenwege
H Denk, HP Dienes, J Düllmann, HP Fischer, O Klinge, W Lierse, KH zum Büschenfelde, U Pfeifer, KH Preisegger, G Ramadori, A Tannapfel, C Wittekind, U Wulfhekel, H Zhou |
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 |
|
Serine proteinase inhibitor therapy in ?1-antitrypsin inhibitor deficiency and cystic fibrosis
G D�ring |
Pediatric Pulmonology | 1999 |
|
The prevalence and clinical significance of α1-antitrypsin deficiency (PiZ) and anca specificities (proteinase 3, BPI) in patients with ulcerative colitis
AN Elzouki, S Eriksson, R Löfberg, L Nässberger, JÖ Wieslander, S Lindgren |
Inflammatory Bowel Diseases | 1999 |
|
Chromosome 14
F Gilbert |
Genetic Testing | 1999 |
|
The Pseudomonas aeruginosa secretory product pyocyanin inactivates alpha1 protease inhibitor: implications for the pathogenesis of cystic fibrosis lung disease
BE Britigan, MA Railsback, CD Cox |
Infection and immunity | 1999 |
|
An Overview of Proteinase Inhibitors
K Hibbetts, B Hines, D Williams |
Journal of Veterinary Internal Medicine | 1999 |
|
Is There a Relationship between Abdominal Aortic Aneurysms and Alpha1-antitrypsin Deficiency (PiZ)?
AN Elzouki, AR Ahlgren, T Länne, B Sonesson, S Eriksson |
European Journal of Vascular and Endovascular Surgery | 1999 |
|
Chronic obstructive pulmonary disease, with and without alpha-1-antitrypsin deficiency: management practices in the U.K
AT Hill, EJ Campbell, AM Ward, RA Stockley |
Respiratory Medicine | 1999 |
|
Ribozyme-mediated specific gene replacement of the α1-antitrypsin gene in human hepatoma cells
I Ozaki, MA Zern, S Liu, DL Wei, RJ Pomerantz, L Duan |
Journal of Hepatology | 1999 |
|
The Prevalence and Clinical Significance of α1-Antitrypsin Deficiency (PiZ) and ANCA Specificities (Proteinase 3, BPI) in Patients with Ulcerative Colitis:
AN Elzouki, S Eriksson, R Löfberg, L Nässberger, J Wieslander, S Lindgren |
Inflammatory Bowel Diseases | 1999 |
|
A Quantification of the Lung Surface Area in Emphysema Using Computed Tomography
HO Coxson, RM Rogers, KP Whittall, Y D'yachkova, PD Paré, FC Sciurba, JC Hogg |
American journal of respiratory and critical care medicine | 1999 |
|
Molecular Biology of the Lung
RA Stockley |
1999 | |
|
The Pseudomonas aeruginosa Secretory Product Pyocyanin Inactivates α1 Protease Inhibitor: Implications for the Pathogenesis of Cystic Fibrosis Lung Disease
EI Tuomanen, BE Britigan, MA Railsback, CD Cox |
Infection and immunity | 1999 |
|
High-Speed Detection of the Two Common α1-Antitrypsin Deficiency Alleles Pi*Z and Pi*S by Real-Time Fluorescence PCR and Melting Curves
C Aslanidis, M Nauck, G Schmitz |
Clinical chemistry | 1999 |
|
α1-Antitrypsin Deficiency: From Genotype to Childhood Disease
N Marcus, JH Teckman, DH Perlmutter |
Journal of Pediatric Gastroenterology & Nutrition | 1998 |
|
Oncostatin M, leukaemia-inhibitory factor and interleukin 6 trigger different effects on alpha1-proteinase inhibitor synthesis in human lung-derived epithelial cells
J Cichy, S Rose-John, J Travis |
Biochemical Journal | 1998 |
|
Use of the 1,2,5-thiadiazolidin-3-one 1,1 dioxide and isothiazolidin-3-one 1,1 dioxide scaffolds in the design of potent inhibitors of serine proteinases
R Kuang, R Venkataraman, S Ruan, WC Groutas |
Bioorganic & Medicinal Chemistry Letters | 1998 |
|
4 α1-Antitrypsin deficiency
S Eriksson, AN Elzouki |
Bailliere's Clinical Gastroenterology | 1998 |
|
Thein VitroEffect of Lithocholic Acid on the Polymerization Properties of PiZ Alpha-1-Antitrypsin
MC Gerbod, S Janciauskiene, JO Jeppsson, S Eriksson |
Archives of Biochemistry and Biophysics | 1998 |
|
Pulmonary Cell Biology
RJ Mason, RG Crystal |
American journal of respiratory and critical care medicine | 1998 |
|
Potent and Specific Inhibition of Human Leukocyte Elastase, Cathepsin G and Proteinase 3 by Sulfone Derivatives Employing the 1,2,5-Thiadiazolidin-3-one 1,1 Dioxide Scaffold
WC Groutas, R Kuang, S Ruan, JB Epp, R Venkataraman, TM Truong |
Bioorganic & Medicinal Chemistry | 1998 |
|
Liver Growth and Repair
A Strain, AM Diehl |
1998 | |
|
Liver Transplant Pathology in Pediatric Metabolic Disorders
R Jaffe |
Pediatric and Developmental Pathology | 1998 |
|
??1-Antitrypsin: Hope on the Horizon for Emphysema Sufferers?
M Schwaiblmair, C Vogelmeier |
Drugs & Aging | 1998 |
|
Regulation of the serine proteinase inhibitor (SERPIN) gene α1-antitrypsin: A paradigm for other SERPINs
K Morgan, NA Kalsheker |
The International Journal of Biochemistry & Cell Biology | 1997 |
|
Intravenous alpha-1-antitrypsin replacement therapy: Case report after one year of treatment
AC Ward, BA Keogh |
Irish Journal of Medical Science | 1997 |
|
Young adults with α1antitrypsin deficiency identified neonatally: their health, knowledge about and adaptation to the high-risk condition
T Sveger, T Thelin, TF McNeil |
Acta Paediatrica | 1997 |
|
NATURAL HISTORY AND PROGNOSIS OF ADVANCED LUNG DISEASE
S Manaker, G Tino |
Clinics in Chest Medicine | 1997 |
|
Case 10-1997
RC Cabot, RE Scully, EJ Mark, WF McNeely, SH Ebeling, LD Phillips, CL Berg, FM Graeme-Cook |
New England Journal of Medicine | 1997 |
|
Evaluation of the effectiveness of different antifoams for an α1 PI solution
MP Flament, P Leterme, T Burnouf, AT Gayot |
International Journal of Pharmaceutics | 1997 |
|
Serine protease inhibitors in patients with chronic viral hepatitis
AN Elzouki, H Verbaan, S Lindgren, A Widell, J Carlson, S Eriksson |
Journal of Hepatology | 1997 |
|
Severe α1-antitrypsin deficiency (PiZ homozygosity) with membranoproliferative glomerulonephritis and nephrotic syndrome, reversible after orthotopic liver transplantation
AN Elzouki, S Lindgren, S Nilsson, B Veress, S Ericksson |
Journal of Hepatology | 1997 |
|
Effects of chronic cigarette smoke inhalation on the development of senile lung in senescence-accelerated mouse
S Teramoto, Y Uejima, T Oka, K Teramoto, T Matsuse, Y Ouchi, Y Fukuchi |
Research in Experimental Medicine | 1997 |
|
Mutation in an alpha1-antitrypsin enhancer results in an interleukin-6 deficient acute-phase response due to loss of cooperativity between transcription factors
K Morgan, G Scobie, P Marsters, N A Kalsheker |
Biochimica et Biophysica Acta | 1997 |
|
Baseline Characteristics of Enrollees in the National Heart, Lung and Blood Institute Registry of α1-Antitrypsin Deficiency
NG McElcaney, JK Stoller, AS Buist, UB Prakash, ML Brantly, MD Schluchter, RD Crystal |
Chest | 1997 |
|
Biosynthesis of α 1 -Proteinase Inhibitor by Human Lung-derived Epithelial Cells
J Cichy, J Potempa, J Travis |
The Journal of biological chemistry | 1997 |
|
α1-Antitrypsin Blocks the Release of Transforming Growth Factor-α from MCF-7 Human Breast Cancer Cells1
J Yavelow, A Tuccillo, SS Kadner, J Katz, TH Finlay |
The Journal of clinical endocrinology and metabolism | 1997 |
|
Molecular biological xxxmethods in diagnosis and treatment of liver diseases
HJ Worman |
Clinical chemistry | 1997 |
|
Molecular Basis, Clinical Consequences and Diagnosis of Alpha-1 Antitrypsin Deficiency
MR Norman, AP Mowat, DC Hutchison |
Annals of Clinical Biochemistry | 1997 |
|
Alpha-1-antitrypsin Deficiency: Biochemistry and Clinical Manifestations
DH Perlmutter |
Annals of Medicine | 1996 |
|
Design, synthesis, and in vitro inhibitory activity toward human leukocyte elastase, cathepsin G, and proteinase 3 of saccharin-derived sulfones and congeners
WC Groutas, JB Epp, R Venkataraman, R Kuang, TM Truong, JJ McClenahan, O Prakash |
Bioorganic & Medicinal Chemistry | 1996 |
|
Role of oxidative mixed-disulfide formation in elastase–serine proteinase inhibitor (serpin) complex
SC Tyagi |
Biochemistry and Cell Biology | 1996 |
|
RECENT PULMONARY OBSERVATIONS IN α1-ANTITRYPSIN DEFICIENCY, PRIMARY BILIARY CIRRHOSIS, CHRONIC HEPATITIS C, AND OTHER HEPATIC PROBLEMS
MJ Krowka |
Clinics in Chest Medicine | 1996 |
|
The Brown Norway rats and the kinin system
J Damas |
Peptides | 1996 |
|
Rapid and Simple Diagnosis of the Two Common α1-Proteinase Inhibitor Deficiency Alleles Pi*Z and Pi*S by DNA Analysis
A Braun, P Meyer, HC †, AA Roscher |
Clinical Chemistry and Laboratory Medicine | 1996 |
|
Les serpines, une classe de protéines fonctionnellement importantes : le paradigme de l'alpha-1-antitrypsine
J Emmerich |
La Revue de Médecine Interne | 1996 |
|
HCV-associated hepatocellular carcinoma without cirrhosis
A El-Refaie, K Savage, S Bhattacharya, S Khakoo, TJ Harrison, M El-Batanony, ES Soliman, S Nasr, N Mokhtar, K Amer, PJ Scheuer, AP Dhillon |
Journal of Hepatology | 1996 |
|
Oxidant-Protease Interaction in the Lung
R Buhl, A Meyer, C Vogelmeier |
Chest | 1996 |
|
A 30-Year Perspective on α1-Antitrypsin Deficiency
S Eriksson |
Chest | 1996 |
|
Alpha1Antitrypsin Deficiency Deaths in the United States From 1979-1991
RJ Browne, DM Mannino, MJ Khoury |
Chest | 1996 |
|
Pathogenesis and Pathology of Liver Disease Associated With α1-Antitrypsin Deficiency
G Massi |
Chest | 1996 |
|
The Endoplasmic Reticulum Degradation Pathway for Mutant Secretory Proteins α1-Antitrypsin Z and S Is Distinct from That for an Unassembled Membrane Protein
JH Teckman, DH Perlmutter |
The Journal of biological chemistry | 1996 |
|
Crucial residues in the carboxy-terminal end of C1 inhibitor revealed by pathogenic mutants impaired in secretion or function
E Verpy, E Couture-Tosi, E Eldering, M Lopez-Trascasa, P Späth, T Meo, M Tosi |
Journal of Clinical Investigation | 1995 |
|
Utilization of transgenic mice in the study of matrix degrading proteinases and their inhibitors
R Khokha, DC Martin, JE Fata |
Cancer and Metastasis Reviews | 1995 |
|
Conceptual advances in the pathogenesis and treatment of childhood metabolic liver disease
J Teckman, DH Perlmutter |
Gastroenterology | 1995 |
|
Isoxazoline derivatives as potential inhibitors of the proteolytic enzymes human leukocyte elastase, cathepsin G and proteinase 3: a structure-activity relationship study
WC Groutas, R Venkataraman, LS Chong, JE Yoder, JB Epp, MA Stanga, EH Kim |
Bioorganic & Medicinal Chemistry | 1995 |
|
The Gabriel-Colman rearrangement in biological systems: design, synthesis and biological evaluation of phthalimide and saccharin derivatives as potential mechanism-based inhibitors of human leukocyte elastase, cathepsin G and proteinase 3
WC Groutas, LS Chong, R Venkataraman, JB Epp, R Kuang, N Houser-Archield, JR Hoidal |
Bioorganic & Medicinal Chemistry | 1995 |
|
The liver in adolescents with α-antitrypsin deficiency
T Sveger, S Eriksson |
Hepatology | 1995 |
|
The PiZ gene of α1-antitrypsin as a determinant of outcome in PR3-ANCA-positive vasculitis
M Segelmark, AN Elzouki, J Wieslander, S Eriksson |
Kidney International | 1995 |
|
What do dysfunctional serpins tell us about molecular mobility and disease?
PE Stein, RW Carrell |
Nature Structural Biology | 1995 |
|
Hepatology
AN McNair, CJ Tibbs, R Williams |
BMJ (Clinical research ed.) | 1995 |
|
Wherefore art thou liver disease associated with alpha- 1 antitrypsin deficiency?
HL Sharp |
Hepatology | 1995 |
|
Biotechnology Annual Review
S Hashida, K Hashinaka, E Ishikawa |
Biotechnology Annual Review | 1995 |
|
Liver Regeneration and Carcinogenesis
EP Sandgren |
Liver Regeneration and Carcinogenesis | 1995 |
|
Clinical Implications of Gene Therapy for Alpha1-Antitrypsin Deficiency
DL Knoell, MD Wewers |
Chest | 1995 |
|
COOH-terminal Substitutions in the Serpin C1 Inhibitor That Cause Loop Overinsertion and Subsequent Multimerization
E Eldering, E Verpy, D Roem, T Meo, M Tosi |
The Journal of biological chemistry | 1995 |
|
Differential Recognition of α 1 -Antitrypsin-Elastase and α 1 -Antichymotrypsin-Cathepsin G Complexes by the Low Density Lipoprotein Receptor-related Protein
W Poller, TE Willnow, J Hilpert, J Herz |
The Journal of biological chemistry | 1995 |
|
Degenerative Diseases of the Retina
RE Anderson, MM LaVail, JG Hollyfield |
1995 | |
|
Relationship between a Mild α 1 Proteinase Inhibitor Deficiency and Respiratory Symptoms in a Family
G Huet-Duvillier, M Balduyck, Y Watrigant, R Sesboue, C Thiebaut, JJ Lafitte, P Degand |
Annals of Clinical Biochemistry | 1995 |
|
CLINICAL MANIFESTATIONS OF ALPHA1-ANTITRYPSIN DEFICIENCY
DH Perlmutter |
Gastroenterology Clinics of North America | 1995 |
|
Modulation of secretory leukoprotease inhibitor gene expression in human bronchial epithelial cells by phorbol ester
M Maruyama, JG Hay, K Yoshimura, CS Chu, RG Crystal |
Journal of Clinical Investigation | 1994 |
|
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 |
|
Interaction of Matrix Metalloproteinases with Serine Protease Inhibitors
SK Mallya, JE Hall, HM Lee, EJ Roemer, SR Simon, LM Golub |
Annals of the New York Academy of Sciences | 1994 |
|
Strong link between the alpha1-antitrypsinPiZallele and Wegener's granulomatosis
AN Elzouki, M Segelmark, J Wieslander, S Eriksson |
Journal of Internal Medicine | 1994 |
|
A young woman with cirrhosis: Autoimmune hepatitis vs. α1-antitrypsin deficiency
AS Lok, MG Ghany, MA Gerber |
Hepatology | 1994 |
|
Hepatocarcinogenesis is the sequel to hepatitis in Z#2 α1-antitrypsin transgenic mice: Histopathological and DNA ploidy studies
SA Geller, WS Nichols, S Kim, T Tolmachoff, S Lee, MJ Dycaico, K Felts, JA Sorge |
Hepatology | 1994 |
|
A family of serine proteases expressed exclusively in myelo-monocytic cells specifically processes the nuclear factor-kappa B subunit p65 in vitro and may impair human immunodeficiency virus replication in these cells
G Franzoso, P Biswas, G Poli, LM Carlson, KD Brown, M Tomita-Yamaguchi, AS Fauci, UK Siebenlist |
Journal of Experimental Medicine | 1994 |
|
Identification and DNA sequence analysis of 15 new alpha 1-antitrypsin variants, including two PI*Q0 alleles and one deficient PI*M allele
JP Faber, W Poller, S Weidinger, M Kirchgesser, R Schwaab, F Bidlingmaier, K Olek |
The American Journal of Human Genetics | 1994 |
|
A lag in intracellular degradation of mutant alpha 1-antitrypsin correlates with the liver disease phenotype in homozygous PiZZ alpha 1-antitrypsin deficiency
Y Wu, I Whitman, E Molmenti, K Moore, P Hippenmeyer, DH Perlmutter |
Proceedings of the National Academy of Sciences | 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 |
|
Diagnostic, preventive, medical and surgical management of alpha1-antitrypsin deficiency in childhood
M Burdelski |
Acta Paediatrica | 1994 |
|
Screening for alpha1-antitrypsin deficiency
T Sveger |
Acta Paediatrica | 1994 |
|
Molecular biology techniques in the diagnosis of monogenic diseases
C Wagener, JT Epplen, H Erlich, H Peretz, P Vihko |
Clinica Chimica Acta | 1994 |
|
Allotypes of α1-antitrypsin in patients with cystic fibrosis, homozygous and heterozygous for deltaF508
G Döring, H Krogh-Johansen, S Weidinger, N Høiby |
Pediatric Pulmonology | 1994 |
|
Abdominal aortic aneurysms and alpha1-antitrypsin
AN Elzouki, S Eriksson |
Journal of Internal Medicine | 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 |
|
Liver transplantation for end-stage liver disease associated with alpha-1-antitrypsin deficiency in children: pretransplant natural history, timing and results of transplantation
F Filipponi, O Soubrane, F Labrousse, D Devictor, O Bernard, J Valayer, D Houssin |
Journal of Hepatology | 1994 |
|
Regulation of the α 1 -Antitrypsin Gene and a Disease-associated Mutation in a Related Enhancer Sequence
NA Kalsheker, K Morgan |
American journal of respiratory and critical care medicine | 1994 |
|
Structure of the Azurocidin, Proteinase 3, and Neutrophil Elastase Genes: Implications for Inflammation and Vasculitis
DE Jenne |
American journal of respiratory and critical care medicine | 1994 |
|
Conformational Changes in Serpins and the Mechanism of α 1 -Antitrypsin Deficiency
RW Carrell, J Whisstock, DA Lomas |
American journal of respiratory and critical care medicine | 1994 |
|
A rapid fluorometric assay for newborn screening of α1-antitrypsin followed by phenotyping of deficient specimens by isoelectric focusing
WC Spence, K Pass, PD Murphy |
Screening | 1994 |
|
Transgenic Animal Technology
GM Monastersky |
Transgenic Animal Technology | 1994 |
|
Three novel mutations of antithrombin inducing high-molecular-mass compounds
J Emmerich, D Vidaud, M Alhenc-Gelas, G Chadeuf, M Gouault-Heilmann, MF Aillaud, M Aiach |
Arteriosclerosis Thrombosis and Vascular Biology | 1994 |
|
Statistical Mechanics, Protein Structure, and Protein Substrate Interactions
S Doniach |
1994 | |
|
Advances in Forensic Haemogenetics: 15th Congress of the International Society for Forensic Haemogenetics (Internationale Gesellschaft für forensische Hämogenetik e.V.), Venezia, 13–15 October 1993
W Bär, A Fiori, U Rossi |
1994 | |
|
Expression and structural analysis of a novel highly inducible gene encoding alpha 1-antitrypsin in rabbit
BK Ray, X Gao, A Ray |
The Journal of biological chemistry | 1994 |
|
Study of the roles of proline 391 and a highly conserved sequence in the carboxyl-terminal region of members of the serpin family in the secretion of alpha 1-proteinase inhibitor
RM Brodbeck, JL Brown |
The Journal of biological chemistry | 1994 |
|
Human genetic therapy
J Harris, K Sikora |
Molecular Aspects of Medicine | 1993 |
|
The hepatic acute-phase proteins alpha 1-antitrypsin and alpha 2-macroglobulin inhibit binding of transferrin to its receptor
I Graziadei, R Kaserbacher, H Braunsteiner, W Vogel |
Biochemical Journal | 1993 |
|
Hepatic gene therapy: adenovirus enhancement of receptor-mediated gene delivery and expression in primary hepatocytes
RJ Cristiano, LC Smith, SL Woo |
Proceedings of the National Academy of Sciences | 1993 |
|
Chemical, biochemical, pharmacokinetic, and biological properties of L-680,833: a potent, orally active monocyclic beta-lactam inhibitor of human polymorphonuclear leukocyte elastase
JB Doherty, SK Shah, PE Finke, CP Dorn, WK Hagmann, JJ Hale, AL Kissinger, KR Thompson, K Brause, GO Chandler |
Proceedings of the National Academy of Sciences | 1993 |
|
PI * S iiyama , a deficiency gene of alpha1-antitrypsin: Evidence for the occurrence in western Japan
I Yuasa, Y Sugimoto, M Ichinose, Y Matsumoto, Y Fukumaki, T Sasaki, K Okada |
The Japanese Journal of Human Genetics | 1993 |
|
: potent inhibitors of human leukocyte elastase
WC Groutas, R Venkataraman, MJ Brubaker, JB Epp, LS Chong, MA Stanga, JJ McClenahan, F Tagusagawa |
Biochimica et Biophysica Acta (BBA) - Protein Structure and Molecular Enzymology | 1993 |
|
The molecular basis of α1-antichymotrypsin deficiency in a heterozygote with liver and lung disease
JP Faber, W Poller, K Olek, U Baumann, J Carlson, B Lindmark, S Eriksson |
Journal of Hepatology | 1993 |
|
Human α 1 -Proteinase Inhibitor Binds to Extracellular Matrix In Vitro
AR Rinehart, S Mallya, SR Simon |
American journal of respiratory cell and molecular biology | 1993 |
|
Pseudomonas aeruginosa as an Opportunistic Pathogen
M Campa, M Bendinelli, H Friedman |
1993 | |
|
Changes of immunoreactivity in ?1-antitrypsin in patients with autoimmune diseases
L Saso, B Silvestrini, R Lahita, CY Cheng |
Inflammation | 1993 |
|
Effects of mutations that alter the Glu264-Lys387 salt bridge on the secretion of alpha-1-proteinase inhibitor
RM Brodbeck, T Samandari, JL Brown |
The Journal of biological chemistry | 1993 |
|
Met 358 to Arg mutation of alpha 1-antitrypsin associated with protein C deficiency in a patient with mild bleeding tendency
D Vidaud, J Emmerich, M Alhenc-Gelas, J Yvart, JN Fiessinger, M Aiach |
Journal of Clinical Investigation | 1992 |
|
Human gene therapy: Present and future
MA Kay, KP Ponder, SL Woo |
Breast Cancer Research and Treatment | 1992 |
|
Purification and characterization by fast-atom-bombardment mass spectrometry of the polymorphonuclear-leucocyte-elastase-generated A alpha (1-21) fragment of fibrinogen from human blood after incubation with calcium ionophore A23187
RS Dewey, JM Liesch, HR Williams, EE Sugg, CA Dolan, P Davies, RA Mumford, G Albers-Schönberg |
Biochemical Journal | 1992 |
|
Adenovirus-mediated transfer of a recombinant human alpha 1-antitrypsin cDNA to human endothelial cells
P Lemarchand, HA Jaffe, C Danel, MC Cid, HK Kleinman, LD Stratford-Perricaudet, M Perricaudet, A Pavirani, JP Lecocq, RG Crystal |
Proceedings of the National Academy of Sciences | 1992 |
|
Potential mechanism-based inhibitors of proteolytic enzymes
WC Groutas, MJ Brubaker, R Venkataraman, JB Epp, N Houser-Archield, LS Chong, JJ McClenahan |
Bioorganic & Medicinal Chemistry Letters | 1992 |
|
The characterization of a mutation of the 3‘ flanking sequence of the α1-antitrypsin gene commonly associated with chronic obstructive airways disease
K Morgan, G Scobie, N Kalsheker |
European Journal of Clinical Investigation | 1992 |
|
Reliable phenotyping of alpha-1-antitrypsin by hybrid isoelectric focusing in an ultranarrow immobilized pH gradient
S Weidinger |
Electrophoresis | 1992 |
|
A general approach toward the design of inhibitors of serine proteinases: inhibition of human leukocyte elastase by substituted dihydrouracils
WC Groutas, H Huang, JB Epp, MJ Brubaker, CE Keller, JJ McClenahan |
Bioorganic & Medicinal Chemistry Letters | 1992 |
|
Mis-sense mutation of α1-antichymotrypsin gene associated with chronic lung disease
W Poller, JP Faber, S Scholz, S Weidinger, K Bartholomé, K Olek, S Eriksson |
The Lancet | 1992 |
|
Gene therapy strategies for pulmonary disease
RG Crystal |
The American Journal of Medicine | 1992 |
|
Enzyme-linked immunosorbent assay for proteolytically inactivated antithrombin-III: Use of sodium dodecyl sulfate to eliminate signal due to intact antithrombin-III
PC Esmon, E Yee |
Analytical Biochemistry | 1992 |
|
Role of disulfide exchange in .alpha.1-protease inhibitor
SC Tyagi, SR Simon |
Biochemistry | 1992 |
|
Understanding and Treating Some Genetic Diseases
M Gibaldi |
The Annals of pharmacotherapy | 1992 |
|
What is Pi (proteinase inhibitor) null or PiQO?: a problem highlighted by the alpha 1 antitrypsin Mheerlen mutation
N Kalsheker, K Hayes, S Weidinger, A Graham |
Journal of medical genetics | 1992 |
|
Biochemistry of Pulmonary Emphysema
C Grassi, J Travis, L Casali, M Luisetti |
1992 | |
|
Secretion of alpha-1-proteinase inhibitor requires an almost full length molecule
RM Brodbeck, JL Brown |
The Journal of biological chemistry | 1992 |
|
Cloning of the human ?2-macroglobulin gene and detection of mutations in two functional domains: the bait region and the thiolester site
W Poller, JP Faber, G Klobeck, K Olek |
Human Genetics | 1992 |
|
Expression of the secretory leukoprotease inhibitor gene in epithelial cells
T Abe, N Kobayashi, K Yoshimura, BC Trapnell, H Kim, RC Hubbard, MT Brewer, RC Thompson, RG Crystal |
Journal of Clinical Investigation | 1991 |
|
α1-antitrypsin deficiency and liver disease
P Birrer, NG McElvaney, LM Chang-Stroman, RG Crystal |
Journal of Inherited Metabolic Disease | 1991 |
|
The cellular basis for liver injury in α1-antitrypsin deficiency
DH Perlmutter |
Hepatology | 1991 |
|
The Discovery and Biologic Properties of Cephalosporin-Based Inhibitors of PMN Elastase
P Davies, BM Ashe, RJ Bonney, C Dorn, P Finke, D Fletcher, WA Hanlon, JL Humes, A Maycock, RA Mumford, MA Navia, EE Opas, S Pacholok, S Shah, M Zimmerman, JB Doherty |
Annals of the New York Academy of Sciences | 1991 |
|
The molecular genetics of ?1 antitrypsin deficiency
Y Wu, RC Foreman |
BioEssays | 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 |
|
Facile redox formation of a 3-substituted maleimide from a 3-substituted N-hydroxysuccinimide
WC Groutas, R Venkataraman, MJ Brubaker, F Tagusagawa |
Tetrahedron Letters | 1991 |
|
α 1 1-Antitrypsin Deficiency: Pathogenesis and Treatment
RG Crystal |
Hospital Practice | 1991 |
|
Molecular cloning and primary structure of rat thyroxine-binding globulin
S Imamura, Y Mori, Y Murata, I Yamamori, Y Miura, Y Oiso, H Seo, N Matsui, S Refetoff |
Biochemistry | 1991 |
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Inhibition of human leukocyte elastase by phosphate esters of N-hydroxysuccinimide and its derivatives: direct observation of phosphorylated enzyme by 31P nuclear magnetic resonance spectroscopy
WC Groutas, R Venkataraman, MJ Brubaker, MA Stanga |
Biochemistry | 1991 |
|
Alpha 1-antitrypsin deficiency, complement activation, and chronic liver disease
ET Littleton, L Bevis, LJ Hansen, M Peakman, AP Mowat, G Mieli-Vergani, D Vergani |
Journal of clinical pathology | 1991 |
|
Journal of Inherited Metabolic Disease
RA Harkness, RJ Pollitt, GM Addison |
Journal of Inherited Metabolic Disease | 1991 |
|
Adenovirus-Mediated Transfer of a Recombinant α1-Antitrypsin Gene to the Lung Epithelium in Vivo
MA Rosenfeld, W Siegfried, K Yoshimura, K Yoneyama, M Fukayama, LE Stier, PK Pääkkö, P Gilardi, LD Stratford-Perricaudet, M Perricaudet, S Jallat, A Pavirani, JP Lecocq, RG Crystal |
Science | 1991 |
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Construction and expression of alpha 1-proteinase inhibitor mutants and the effects of these mutations on secretion of the variant inhibitors
AA McCracken, KB Kruse, J Valentine, C Roberts, TZ Yohannes, JL Brown |
The Journal of biological chemistry | 1991 |
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Siiyama (serine 53 (TCC) to phenylalanine 53 (TTC)). A new alpha 1-antitrypsin-deficient variant with mutation on a predicted conserved residue of the serpin backbone
K Seyama, T Nukiwa, K Takabe, H Takahashi, K Miyake, S Kira |
The Journal of biological chemistry | 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 |
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Amyloid-beta peptide, substance P, and bombesin bind to the serpin-enzyme complex receptor
G Joslin, JE Krause, AD Hershey, SP Adams, RJ Fallon, DH Perlmutter |
The Journal of biological chemistry | 1991 |
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Expression of the beta-hexosaminidase alpha subunit gene with the four-base insertion of infantile Jewish Tay-Sachs disease
J Nishimoto, A Tanaka, E Nanba, K Suzuki |
The Journal of biological chemistry | 1991 |
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Molecular biology and respiratory disease. 7. The alpha 1 antitrypsin gene and chronic lung disease
N Kalsheker, K Morgan |
Thorax | 1990 |
|
Liver Disorders in Childhood
AP Mowat |
Liver Disorders in Childhood | 1979 |
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