The role of proteases, endoplasmic reticulum stress andSERPINA1heterozygosity in lung disease and α-1 anti-trypsin deficiency

A Novel Proteolytic Cascade Generates an Extracellular Matrix-Derived Chemoattractant in Chronic Neutrophilic Inflammation

(2014) A. Gaggar et al.   JOURNAL OF IMMUNOLOGY

Tumor Necrosis Factor–α rs361525 Polymorphism Is Associated with Increased Local Production and Downstream Inflammation in Chronic Obstructive Pulmonary Disease

(2010) Elizabeth Sapey et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Alpha-1-Antitrypsin Deficiency: Importance of Proteasomal and Autophagic Degradative Pathways in Disposal of Liver Disease–Associated Protein Aggregates

(2010) David H. Perlmutter   Annual Review of Medicine

α; 1 -Antitrypsin Protease Inhibitor MZ Heterozygosity Is Associated With Airflow Obstruction in Two Large Cohorts

(2010) Inga-Cecilie Sørheim et al.   CHEST

Augmentation Therapy for Alpha-1 Antitrypsin Deficiency—Not Enough Evidence to Support its Use Yet!

(2010) Cormac McCarthy et al.   COPD-Journal of Chronic Obstructive Pulmonary Disease

Preclinical Evaluation of a Recombinant Adeno-Associated Virus Vector Expressing Human Alpha-1 Antitrypsin Made Using a Recombinant Herpes Simplex Virus Production Method

(2010) Jeffrey D. Chulay et al.   HUMAN GENE THERAPY

α-1 Antitrypsin regulates human neutrophil chemotaxis induced by soluble immune complexes and IL-8

(2010) David A. Bergin et al.   JOURNAL OF CLINICAL INVESTIGATION

Evidence for Unfolded Protein Response Activation in Monocytes from Individuals with  -1 Antitrypsin Deficiency

(2010) T. P. Carroll et al.   JOURNAL OF IMMUNOLOGY

ALPHA-1-ANTITRYPSIN DEFICIENCY IN A 78-YEAR-OLD WOMAN WITH ISOLATED LIVER CIRRHOSIS

(2010) Nathalie Voide et al.   JOURNAL OF THE AMERICAN GERIATRICS SOCIETY

Clearing Conformational Disease

(2010) R. N. Sifers   SCIENCE

An Autophagy-Enhancing Drug Promotes Degradation of Mutant  1-Antitrypsin Z and Reduces Hepatic Fibrosis

(2010) T. Hidvegi et al.   SCIENCE

Generation of Transgene-Free Lung Disease-Specific Human Induced Pluripotent Stem Cells Using a Single Excisable Lentiviral Stem Cell Cassette

(2010) Aba Somers et al.   STEM CELLS

Establishment of lentiviral-vector-mediated model of human alpha-1 antitrypsin delivery into hepatocyte-like cells differentiated from mesenchymal stem cells

(2010) Mahboobe Ghaedi et al.   TISSUE & CELL

CT Scan Appearance, Densitometry, and Health Status in Protease Inhibitor SZ α 1 -Antitrypsin Deficiency

(2009) Jayne Holme et al.   CHEST

Phenotypic Differences in Alpha 1 Antitrypsin-Deficient Sibling Pairs May Relate to Genetic Variation

(2009) Alice M. Wood et al.   COPD-Journal of Chronic Obstructive Pulmonary Disease

Augmentation Therapy for α1Antitrypsin Deficiency: A Meta-Analysis

(2009) Kenneth R. Chapman et al.   COPD-Journal of Chronic Obstructive Pulmonary Disease

Anti-apoptotic effects of Z  1-antitrypsin in human bronchial epithelial cells

(2009) C. M. Greene et al.   EUROPEAN RESPIRATORY JOURNAL

Exploring the role of CT densitometry: a randomised study of augmentation therapy in  1-antitrypsin deficiency

(2009) A. Dirksen et al.   EUROPEAN RESPIRATORY JOURNAL

Single nucleotide polymorphism-mediated translational suppression of endoplasmic reticulum mannosidase I modifies the onset of end-stage liver disease in alpha1-antitrypsin deficiency

(2009) Shujuan Pan et al.   HEPATOLOGY

Genetic Modifiers of Liver Disease in Cystic Fibrosis

(2009) Jaclyn R. Bartlett   JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION

Selenoprotein S/SEPS1 Modifies Endoplasmic Reticulum Stress in Z Variant α1-Antitrypsin Deficiency

(2009) Emer Kelly et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Decreased Levels of Secretory Leucoprotease Inhibitor in the Pseudomonas-Infected Cystic Fibrosis Lung Are Due to Neutrophil Elastase Degradation

(2009) S. Weldon et al.   JOURNAL OF IMMUNOLOGY

Alpha1-Antitrypsin Deficiency

(2009) Edwin K. Silverman et al.   NEW ENGLAND JOURNAL OF MEDICINE

Sustained transgene expression despite T lymphocyte responses in a clinical trial of rAAV1-AAT gene therapy

(2009) M. L. Brantly et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The liver in 30-year-old individuals with alpha1-antitrypsin deficiency

(2009) E. Bernspång et al.   SCANDINAVIAN JOURNAL OF GASTROENTEROLOGY

Chronic obstructive pulmonary disease: towards pharmacogenetics

(2009) Alice M Wood et al.   Genome Medicine

A Genome-Wide Association Study in Chronic Obstructive Pulmonary Disease (COPD): Identification of Two Major Susceptibility Loci

(2009) Sreekumar G. Pillai et al.   PLoS Genetics

Matrix Metalloproteinase-8 Facilitates Neutrophil Migration through the Corneal Stromal Matrix by Collagen Degradation and Production of the Chemotactic Peptide Pro-Gly-Pro

(2008) Michelle Lin et al.   AMERICAN JOURNAL OF PATHOLOGY

Age of SERPINA1 Gene PI Z Mutation: Swedish and Latvian Population Analysis

(2008) B. Lace et al.   ANNALS OF HUMAN GENETICS

Autophagy: Basic Principles and Relevance to Disease

(2008) Mondira Kundu et al.   Annual Review of Pathology-Mechanisms of Disease

Autophagy: A lysosomal degradation pathway with a central role in health and disease

(2008) Eeva-Liisa Eskelinen et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

α1-Antitrypsin Augmentation Therapy for PI*MZ Heterozygotes

(2008) Robert A. Sandhaus et al.   CHEST

The Atg8 and Atg12 ubiquitin-like conjugation systems in macroautophagy. ‘Protein Modifications: Beyond the Usual Suspects’ Review Series

(2008) Jiefei Geng et al.   EMBO REPORTS

Persistent episomal transgene expression in liver following delivery of a scaffold/matrix attachment region containing non-viral vector

(2008) O Argyros et al.   GENE THERAPY

Elafin, an Elastase-specific Inhibitor, Is Cleaved by Its Cognate Enzyme Neutrophil Elastase in Sputum from Individuals with Cystic Fibrosis

(2008) Nicolas Guyot et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Activation of the Epidermal Growth Factor Receptor (EGFR) by a Novel Metalloprotease Pathway

(2008) David A. Bergin et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Alpha-1 antitrypsin deficiency: A conformational disease associated with lung and liver manifestations

(2008) C. M. Greene et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Recombinant AAV Serotype and Capsid Mutant Comparison for Pulmonary Gene Transfer of α-1-Antitrypsin Using Invasive and Noninvasive Delivery

(2008) Rejean Liqun Wang et al.   MOLECULAR THERAPY

Alpha-1-antitrypsin aerosolised augmentation abrogates neutrophil elastase-induced expression of cathepsin B and matrix metalloprotease 2 in vivo and in vitro

(2008) P Geraghty et al.   THORAX

IL10Polymorphisms Are Associated with Airflow Obstruction in Severe α1-Antitrypsin Deficiency

(2007) Dawn L. DeMeo et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Proteinases and signalling: pathophysiological and therapeutic implications via PARs and more

(2007) R Ramachandran et al.   BRITISH JOURNAL OF PHARMACOLOGY

Pneumococcal Vaccination for Patients With COPD

(2007) Jeremy G. Schenkein et al.   CHEST