Alpha-1 Related Publications by A1F Funded Investigators
Abusriwil, Hatem
Alpha-1 Antitrypsin replacement therapy: current status
Current Opinions in Pulmonary Medicine
Adair-Kirk, Tracy
Neutrophil elastase cleaves Laminin-332 (laminin-5) generating peptides that are chemotactic for neutrophils
Journal of Biological Chemistry
Arvan, Peter
Endoplasmic reticulum (ER) chaperone regulation and survival of cells compensating for deficiency in the ER stress response kinase, PERK
Journal of Biological Chemistry
The trafficking of alpha-1 antitrypsin, a post-Golgi secretory pathway marker, in INS-1 pancreatic beta cells
Journal of Biological Chemistry
Bezerra, Jorge
High Prevalence of alpha-1 antitrypsin heterozygosity in children with chronic liver disease
Journal of Pediatric Gastroenterology
Hepatocyte transplantation.
Advancing Biology and treating children
Clinics in Liver Disease
Blumental-Perry, Anna
Phosphatidylinositol 4-phosphate formation at ER exit sites regulates ER export
Development Cell
Bowler, Russell
Cigarette smoke impairs clearance of apoptotic cells through oxidant-dependent activation of RhoA
American Journal of Repiratory Care Medicine
Lovastatin enhances clearance of apoptotic cells (efferocytosis) with implications for chronic obstructive pulmonary disease
The Journal of Immunology
Brantly, Mark
Sustained transgene expression despite T lymphocyte responses in a clinical trial of rAAV1-AAT gene therapy
Proceedings of the National Academy of Sciences
Single nucleotide polymorphism-mediated translational suppression of endoplasmic reticulum mannosidase 1 modifies the onset of end-stage liver disease in Alpha-1 Antitrypsin Deficiency
Hepatology
Heritability of lung function in severe Alpha-1 Antitrypsin Deficiency
Human Heredity
Human alpha-1 antitrypsin therapy induces fatal anaphylaxis in non-obese diabetic mice
Clinical and Experimental Immunology
Endothelial alpha-1 antitrypsin attenuates cigarette smoke induced apoptosis in vitro
COPD
Alpha-1 Antitrypsin aerosolized augmentation abrogates neutrophil elastase-induced expression of cathepsin B and matrix metalloprotease 2 in vivo and in vitro
Thorax
Primary care diagnosis of Alpha-1 Antitrypsin Deficiency: issues and opportunities
Cleveland Clinic Journal of Medicine
Prevalence of Alpha-1 Antitrypsin Deficiency in poorly controlled asthma-results from the ALA-ACRC low-dose theophylline trial
Journal of Asthma
IL10 polymorphisms are associated with airflow obstruction in severe Alpha-1 Antitrypsin Deficiency
American Journal of Respiratory Cell and Molecular Biology
In vivo post-transcriptional gene silencing of alpha-1 antitrypsin by adeno-associated virus vectors expressing siRNA
Laboratory Investigation
Determinants of airflow obstruction in severe Alpha-1 Antitrypsin Deficiency
Thorax
Multi-Center study: the biochemical efficacy, safety and tolerability of a new alpha-1 proteinase inhibitor, Zemaira
COPD
Efficient and accurate approaches to the laboratory diagnosis of Alpha-1 Antitrypsin Deficiency: The promise of early diagnosis and intervention
Clinical Chemistry
Phase 1 trial of intramuscular injection of a recombinant adeno-associated virus serotype 2 alpha-1 antitrypsin (AAT) vector in AAT deficient adults
Human Gene Therapy
Does the heterozygous state of Alpha-1 Antitrypsin Deficiency have a role in chronic liver diseases? Interim results of a large case-control study
Journal of Pediatric Gastroenterology
Genetic testing of minors for Alpha-1 Antitrypsin Deficiency
Archives of Pediatrics and Adolescent Medicine
Cytotoxic concentrations of alpha-defensins in the lungs of individuals with Alpha-1 Antitrypsin Deficiency and moderate to severe lung disease
Cytokine
Antibody response to aerosolized transgenic human alpha-1 antitrypsin
New England Journal of Medicine
Genetic testing for Alpha-1 Antitrypsin Deficiency
Genetics in Medicine
Z alpha-1 antitrypsin polymerizes in the lung and acts as a neutrophil chemoattractant
CHEST
Phase 1 trial of intramuscular injection of a recombinant adeno-associated virus alpha-1 antitrypsin (rAAV2-CB-hAAT) gene vector to AAT deficient adults
PMID
Role of human neutrophil peptides in lung inflammation associated with Alpha-1 Antitrypsin Deficiency
Lung Cellular and Molecular Physiology
Asthma features in severe Alpha-1 Antitrypsin Deficiency: experience of the National Heart, Lung, and Blood Institute Registry
CHEST
Alpha-1 antitrypsin: not just an antiprotease: extending the half-life of a natural anti-inflammatory molecule by conjugation with polyethylene glycol
American Journal of Respiratory Cell and Molecular Biology
Biochemical efficacy and safety of a new pooled human plasma alpha-1 antitrypsin, Respitin
CHEST
Formation and current results of a patient-organized registry for Alpha-1 Antitrypsin Deficiency
CHEST
Molecular mechanisms of alpha-1 antitrypsin null alleles
Respiratory Medicine
Lung neutrophil burden correlates with increased pro-inflammatory cytokines and decreased lung function in individuals with Alpha-1 Antitrypsin Deficiency
PMID
Endothelial nitric oxide synthase as a potential susceptibility gene in the pathogenesis of emphysema in Alpha-1 Antitrypsin Deficiency
American Journal of Respiratory Cell and Molecular Biology
Efficient in vivo catheter-based pericardial gene transfer mediated by adenoviral vectors
Clinical Cardiology
Brenner, David
Genomics of liver fibrosis and cirrhosis
Seminars in Liver Disease
Genetic polymorphisms and the progression of liver fibrosis: a critical appraisal
Hepatology
Brodsky, Jeff
One step at a time: endoplasmic reticulum-associated degradation
Nature Reviews Molecular Cellular Biology
The protective and destructive roles played by molecular chaperones during ERAD
Biochemical Journal
Molecular pathogenesis of Alpha-1 Antitrypsin Deficiency associated liver disease: a meeting review
Hepatology
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 alpha-1 porteinase inhibitor (A1 PiZ) and another for aggregates of A1 PiZ
Molecular Biology of the Cell
Yeast as a model system to investigate protein conformational diseases
Methods in Molecular Biology
ADD66, a gene involved in the endoplasmic reticulum-associated degradation of alpha-1 antitrypsin Z in yeast, facilitates proteasome activity and assembly
Molecular Biology of the Cell
Buchanan, Robert
Analysis of male residents in community nursing facilities: comparisons of Veterans Health Administration residents to other residents
Journal of Rehabilitation Research Development
Campos, Michael
Safety and efficacy of alpha-1 antitrypsin augmentation therapy in the treatment of patients with Alpha-1 Antitrypsin Deficiency
Biologics
Exacerbations in subjects with Alpha-1 Antitrypsin Deficiency receiving augmentation therapy
Respiratory Medicine
Effects of a disease management program in individuals with Alpha-1 Antitrypsin Deficiency
COPD
Clinical characteristics of subjects with symptoms of Alpha-1 Antitrypsin Deficiency older than 60 years
CHEST
Influenza vaccination in subjects with Alpha-1 Antitrypsin Deficiency
CHEST
Trends in the diagnosis of symptomatic patients with Alpha-1 Antitrypsin Deficiency between 1968 and 2003
CHEST
Cantor, Jerome
Synergistic effect of hydrogen peroxide and elastase on elastic fiber injury in vitro
Experimental Biology and Medicine
Aerosolized hyaluronan limits airspace enlargement in a mouse model of cigarette smoke-induced pulmonary emphysema
Experimental Lung Research
Carroll, Tomas
Selenoprotein S/SEPS1 modifies endoplasmic reticulum stress in Z variant Alpha-1 Antitrypsin Deficiency
Journal of Biological Chemistry
Viral inhibition of IL-1 and neutrophil elastase-induced inflammatory responses in bronchial epithelial cells
Journal of Immunology
Neutrophil elastase up-regulates interleukin-8 via toll-like receptor 4
FEBS Letters
Coors, Marylin
Anxiety related to genetic testing for Alpha-1 Antitrypsin Deficiency and cystic fibrosis in COPD and/or bronchiectasis
Community Genetics
Cosio, Manuel
Alpha-1 antitrypsin determines the pattern of emphysema and function in tobacco smoke-exposed mice: parallels with human disease
American Journal of Respiratory and Critical Care Medicine
Cox, Diane
Prenatal diagnosis for Alpha-1 Antitrypsin Deficiency
Prenatal Diagnosis
Coxson, Harvey
Quantitative chest tomography in COPD research: a chairman's summary
Proceedings of the American Thoracic Society
Cruz, Pedro
Recombinant AAV serotype and capsid mutant comparison for pulmonary gene transfer of alpha-1 antitrypsin using invasive and noninvasive deliver
Molecular Therapy
Removal of envelope protein-free retroviral vectors by anion-exchange chromatography to improve product quality
Journal of Separation Science
The promise of gene therapy for the treatment of Alpha-1 Antitrypsin Deficiency
Pharmacogenomics
In vivo post-transcriptional gene silencing of alpha-1 antitrypsin by adeno-associated virus vectors expressing siRNA
Laboratory Investigation
Scaleable purification process for gene therapy retroviral vectors
Journal of Genetic Medicine
Alpha-1 antitrypsin inhibits caspase-3 activity, preventing lung endothelial cell apoptosis
American Journal of Pathology
Purification of retroviral vectors for clinical application: biological implications and technological challenges
Journal of Biotechnology
A novel antiapoptotic role for alpha-1 antitrypsin in the prevention of pulmonary emphysema
American Journal of Respiratory and Critical Care Medicine
D'Armiento, Jeanine
Transgenic expression of matrix metalloproteinase-9 causes adult-onset emphysema in mice associated with the loss of alveolar elastin
American Journal of Lung Cellular and Molecular Physiology
De Serres, Fred
Long-term augmentation therapy with alpha-1 antitrypsin in an MZ-AAT severe persistent asthma
Monaldi Archives of Chest Disease
PI S and PI Z Alpha-1 Antitrypsin Deficiency worldwide. A review of existing genetic epidemiological data
Monaldi Archives of Chest Disease
Estimating the risk for Alpha-1 Antitrypsin Deficiency among COPD patients: evidence supporting targeted screening
COPD
Estimated numbers and prevalence of PI S and PI Z alleles of Alpha-1 Antitrypsin Deficiency in Asia
European Respiratory Journal
Estimated numbers and prevalence of PI S and PI Z alleles of Alpha-1 Antitrypsin Deficiency in European countries
European Respiratory Journal
Alpha-1 Antitrypsin Deficiency in Italy: Regional differences of the PI S and PI Z deficiency alleles
Monaldi Archives of Chest Disease
High prevalence of alpha-1 antitrypsin phenotypes in viral hepatitis B infected patients in Iran
Hepatology Research
Alpha-1 antitrypsin phenotypes and HLA-B27 typing in uveitis patients in southeast Iran
Clinical Biochemistry
Health implications of Alpha-1 Antitrypsin Deficiency in Sub-Sahara African countries and their emigrants in Europe and the New World
Genetics Medicine
Alpha-1 antitrypsin and fibromyalgia: new data in favor of the inflammatory hypothesis of fibromyalgia
Medical Hypothesis
PI S and PI Z Alpha-1 Antitrypsin Deficiency: estimated prevalence and number of deficient alleles in Spain
Medicina Clinica
Alpha-1 antitrypsin replacement therapy controls fibromyalgia symptoms in 2 patients with PI ZZ Alpha-1 Antitrypsin Deficiency
Journal of Rheumatology
Alpha-1 Antitrypsin Deficiency is not a rare disease but a disease that is rarely diagnosed
Environmental Health
Perspectives
Genetic epidemiology of Alpha-1 Antitrypsin Deficiency in North America and Australia/New Zealand: Australia, Canada, New Zealand and the United States of America
Clinical Genetics
Environmental, occupational, and genetic risk factors for Alpha-1 Antitrypsin Deficiency
Environmental Health
Perspectives
Genetic epidemiology of Alpha-1 Antitrypsin Deficiency in southern Europe: France, Italy, Portugal and Spain
Clinical Genetics
Worldwide racial and ethnic distribution of Alpha-1 Antitrypsin Deficiency: summary of an analysis of published genetic epidemiologic surveys
CHEST
Deep-Sharma, Amar
Murine embryonic stem cell-derived hepatic progenitor cells engraft in recipient livers with limited capacity of liver tissue formation
Cell Transplantation
DeMeo, Dawn
Heritability of lung function in severe Alpha-1 Antitrypsin Deficiency
Human Heredity
Determinants of airflow obstruction in severe Alpha-1 Antitrypsin Deficiency
Thorax
The SERPINE2 gene is associated with chronic obstructive pulmonary disease
Proceedings of the American Thoracic Society
Predictors of survival in severe, early onset COPD
Chest
Alpha-1 Antitrypsin Deficiency 2: genetic aspects of Alpha-1 Antitrypsin Deficiency: phenotypes and genetic modifiers of emphysema risk
Thorax
Eissa, N. Tony
Endothelial nitric oxide synthase as a potential susceptibility gene in the pathogenesis of emphysema in Alpha-1 Antitrypsin Deficiency
American Journal of Respiratory Cell and Molecular Biology
Fanos, Joanna
The lion, the witch and the wardrobe: impact on siblings of individuals with AAT Deficiency
American Journal of Medical Genetics
Flotte, Terence
Sustained transgene expression despite T lymphocyte responses in a clinical trial of rAAV1-AAT gene therapy
Proceedings of the National Academy of Science
Recombinant AAV serotype and capside mutant comparison for pulmonary gene transfer of alpha-1 antitrypsin using invasive and noninvasive delivery
Molecular Therapy
The promise of gene therapy for the treatment of Alpha-1 Antitrypsin Deficiency
Pharmacogenomics
In vivo post-transcriptional gene silencing of alpha-1 antitrypsin by adeno-associated virus vectors expressing siRNA
Laboratory Investigation
Alpha-1 antitrypsin inhibits caspase-3 activity , preventing lung endothelial cell apoptosis
American Journal of Pathology
Therapeutic level of functional human alpha-1 antitrypsin (hAAT) secreted from murine muscle transduced by adeno-associated virus (rAAV1) vector
Journal of Genetics Medicine
A novel antiapoptotic role for alpha-1 antitrypsin in the prevention of pulmonary emphysema
American Journal of Respiratory and Critical Care Medicine
Adeno-associated virus-based gene therapy for inherited disorders
Pediatric Research
Recent developments in recombinant AAV-mediated gene therapy for lung diseases
Current Gene Therapy
Phase 1 trial of intramuscular injection of a recombinant adeno-associated virus alpha-1 antitrypsin (rAAV2-CB-hAAT) gene vector to AAT-deficient adults
Human Gene Therapy
Intramuscular administration of recombinant adeno-associated virus 2 alpha-1 antitrypsin (rAAV-SERPINA1) vectors in a nonhuman primate model: safety and immunologic aspects
Molecular Therapy
Recombinant adeno-associated virus gene therapy for cystic fibrosis and Alpha-1 Antitrypsin Deficiency
Chest
Gershenson, Anne
Short-lived protease serpin complexes: partial disruption of the rat trypsin active site
Protein Science
Conformational distributions of protease-serpin complexes: a partially translocated complex
Biochemistry
Gildea, Thomas
Lung volume reduction surgery in patients with emphysema and Alpha-1 Antitrypsin Deficiency
Annals of Thoracic Surgery
Cost effectiveness of population screening for Alpha-1 Antitrypsin Deficiency: a decisions analysis
COPD
Cost effectiveness analysis of augmentation therapy for severe Alpha-1 Antitrypsin Deficiency
American Journal of Respiratory and Critical Care Medicine
Emphysema in nonsmokers: Alpha-1 Antitrypsin Deficiency and other causes
Cleveland Clinic Journal of Medicine
Greene, Catherine
Gene targeted therapeutics for liver disease in Alpha-1 Antitrypsin Deficiency
Biologics
Selenoprotein S/SEPS1 modifies endoplasmic reticulum stress in Z variant Alpha-1 Antitrypsin Deficiency
Journal of Biological Chemistry
Z alpha-1 antitrypsin polymerizes in the lung and acts as a neutrophil chemoattractant
CHEST
Local impairment of anti-neutrophil elastase capacity in community-acquired pneumonia
Journal of Infectious Diseases
Guerra, Stefano
A longitudinal study of alpha-1 antitrypsin phenotypes and decline in FEV1 in a community population
CHEST
Hersh, Craig
A genome-wide association study in chronic obstructive pulmonary disease: identification of two major susceptibility loci
PLOS Genetics
Analysis of exonic elastin variants in severe, early-onset chronic obstructive pulmonary disease
American Journal of Respiratory Cell and Molecular Biology
Genetic Linkage and association analysis of COPD-related traits on chromosome 8p
COPD
Predictors of survival in severe, early-onset COPD
CHEST
Hidvegi, Tunda
Regulator of G Signaling 16 is a marker for the distinct endoplasmic reticulum stress state associated with aggregated mutant alpha-1 antitrypsin Z in the classical form of Alpha-1 Antitrypsin Deficiency
Journal of Biological Chemistry
Intracellular inclusions containing mutant alpha-1 antitrypsin Z are propagated in the absence of autophagic activity
Journal of Biologic Chemistry
Accumulation of mutant alpha-1 antitrypsin Z in the endoplasmic reticulum activates caspases-4 and 12, NFkappaB, and BAP31 but not the unfolded protein response
Journal of Biologic Chemistry
Hoshino, Yuma
The development of emphysema in cigarette smoke-exposed mice is strain dependent
American Journal of Respiratory and Critical Care Medicine
Houghton, A. McGarry
Epithelial cell apoptosis causes acute lung injury masquerading as emphysema
American Journal of Respiratory Cell and Molecular Biology
Janciauskiene, Sabina
Oxidized alpha-1 antitrypsin stimulates the release of monocyute chemotactic protein-1 from lung epithelial cells: potential role in emphysema
American Journal of Lung Cellular and Molecular Physiology
Long-term augmentation therapy with alpha-1 antitrypsin in MZ-AAT severe persistent emphysema
Monaldi Archives of Chest Disease
New Findings in PiZZ Alpha-1 Antitrypsin Deficiency related panniculitis. Demonstration of skin polymers and high dosing requirements of intravenous augmentation therapy
Dermatology
Alpha-1 antitrypsin inhibits epithelial Na+ transport in vitro and in viv
American Journal of Respiratory Cell and Molecular Biology
An association between Type 2 diabetes and Alpha-1 Antitrypsin Deficiency
Diabetes Medicine
Alpha-1 antitrypsin inhibits the activity of the matriptase catalytic domain in vitro
American Journal of Respiratory Cell and Molecular Biology
Screening for Alpha-1 Antitrypsin Deficiency in Lithuanian patients with COPD
Respiratory Medicine
Endotoxin receptor CD14 in PiZ in Alpha-1 Antitrypsin Deficiency individual
Respiratory Research
Hereditary Alpha-1 Antitrypsin Deficiency can cause COPD. Substitution treatment seems to result in fewer infections and prolonged survival
Lakartidningen
TNF-alpha induced self expression in human lung endothelial cells is inhibited by native and oxidized alpha-1 antitrypsin
International Journal
Alpha-1 antitrypsin regulates CD14 expression and soluble CD14 levels in human monocytes in vitro
International Journal of Biochemistry and Cell Biology
Alpha-1 antitrypsin, old dog, new tricks. Alpha-1 antitrypsin exerts in vitro anti-inflammatory activity in human monocytes by elevating cAMP
Journal of Biological Chemistry
Plasma levels of alpha-1 antichymotrypsin and secretory leukocyte proteinase inhibitor in healthy and chronic obstructive pulmonary disease subjects with and without severe Alpha-1 Antitrypsin Deficiency
BMC Pulmonary Medicine
Do native and polymeric alpha-1 antitrypsin activate human neutrophils in vitro?
CHEST
C-36 peptide, a degradation product of alpha-1 antitrypsin, modulates human monocyte activation through LPS signaling pathways
International Journal of Biochemistry and Cell Biology
Alpha-1 antitrypsin inhibits Moraxella catarrhalis MID protein-induced tonsillar B cell proliferation and IL-6 release
Immunology Letters
Polymers of Z alpha-1 antitrypsin co-localize with neutrophils in emphysematous alveoli and are chemotactic in vivo
American Journal of Pathology
Polymerized alpha antitrypsin is present on lung vascular endothelium. New insights into the biological significance of alpha antitrypsin polymerization
Histopathology
ELISA for specific detection of PiZ-related Alpha-1 Antitrypsin Deficiency
Clinical Chemistry
Alpha-1 antitrypsin and its C-terminal fragment attenuate effects of degranulated neutrophil-conditioned medium on lung cancer HCC cells, in vitro
Cancer Cell International
Differential detection of PAS-positive inclusions formed by the Z, Siiyama, and Mmalton variants of alpha-1 antitrypsin
Hepatology
Inhibition of lipopolysaccharide-mediated human monocyte activation, in vitro by alpha-1 antitrypsin
Biochemical and Biophysical Research Communications
Concentration-dependent effects of native and polymerized alpha-1 antitrypsin on primary human monocytes, in vitro
BMC Cell Biology
Increased plasma levels of serine proteinase inhibitors in lung cancer patients
Anticancer Research
Divergent effects of alpha-1 antitrypsin on neutrophil activation in vitro
Biochemical and Biophysical Research Communications
Multiple effects of alpha-1 antitrypsin on breast carcinoma MDA-MB 468 cell growth and invasiveness
European Journal of Cancer Prevention
Detection of circulating endothelial cell polymers of Z and wild type alpha-1 antitrypsin by a monoclonal antibody
Journal of Biological Chemistry
Fibrillogenic C-terminal fragment of alpha-1 antitrypsin activates human monocytes via oxidative mechanisms
Cell and Tissue Research
Conformational properties of serine proteinase inhibitors (serpins) confer multiple pathophysiological roles
International Journal of Biochemistry, Biophysics and molecular biology
Alpha-1 antitrypsin is present in the specific granules of human eosinophilic granulocytes
Clinical and Experimental Allergy
Atherogenic properties of human monocytes induced by the carboxyl terminal proteolytic fragment of alpha-1 antitrypsin
Atherosclerosis
Human monocyte activation by cleaved form of alpha-1 antitrypsin involvement in the phagocytic pathway
European Journal of Biochemistry
Effects of fibrillar C-terminal fragment of cleaved alpha-1 antitrypsin on cholesterol homeostasis in HepG2 cells
Hepatology
Kheradmand, Farrah
A protease-activated pathway underlying Th cell type 2 activation andallergic lung disease
Journal of Immunology
Kotton, Darrell
Sustained expression of alpha-1 antitrypsin after transplantation of manipulated hematopoietic stem cells
American Journal of Respiratory Cell Molecular Biology
Li, Chengwen
Therapeutic level of functional human alpha-1 antitrypsin (hAAT) secreted from murine muscle transduced by adeno-associated virus (rAAV1) vector
Journal of Gene Medicine
Luke, Cliff
Identification and activity of a lower eukaryotic serine proteinase inhibitor (serpin) from Cyanea capillata:
Biochemistry
Mahadeva, Ravi
Oxidized alpha-1 antitrypsin stimulates the release of monocyte chemotactic protein-1 from lung epithelial cells: potential role in emphysema
American Journal of Lung Cellular and Molecular Physiology
Small-molecule peptides inhibit Z alpha-1 antitrypsin polymerization
Journal of Cellular and Molecular Medicine
An association between Type 2 diabetes and Alpha-1 Antitrypsin Deficiency
Diabetic Medicine
Identification of a 4-mer peptide inhibitor that effectively blocks the polymerization of pathogenic Z alpha-1 antitrypsin
American Journal of Respiratory Cell and Molecular Biology
Polymers of Z alpha-1 antitrypsin co-localize with neutrophils in emphysematous alveoli and are chemotactic in vivo
American Journal of Pathology
Inhibiting polymerization: new therapeutic strategies for Z alpha-1 antitrypsin related emphysema
American Journal of Cell and Molecular Biology
Targeting a surface cavity of alpha-1 antitrypsin to prevent conformational disease
Journal of Biological Chemistry
Alpha-1 Antitrypsin Deficiency, liver disease and emphysema
International
Journal of Biochemistry and Cell Biology
Alpha-1 antitrypsin polymerization and the serpinopathies: pathobiology and prospects for therapy
Journal of Clinical Investigation
Polymers of alpha-1 antitrypsin are chemotactic for human neutrophils: a new paradigm for the pathogenesis of emphysema
American Journal of Respiratory Cell and Molecular Biology
6-mer peptide selectively anneals to a pathogenic serpin conformation and blocks polymerization. Implications for the prevention of Z alpha-1 antitrypsin related cirrhosis
Journal of Biological Chemistry
Characterization of a new variants of alpha-1 antitrypsin E (Johannesburg) (H15N) in association with asthma
Human Mutation
A novel polymorphism (471CT) in alpha-1 antitrypsin in a patient with asthma
Human Mutation
Association of alpha-1 antichymotrypsin deficiency with milder lung disease in patients with cystic fibrosis
Thorax
McElvaney, Noel Gerald
Gene targeted therapeutics for liver disease in Alpha-1 Antitrypsin Deficiency
Biologics
Selenoprotein S/SEPS1 modifies endoplasmic reticulum stress in Z variant Alpha-1 Antitrypsin Deficiency
Journal of Biological Chemistry
Heritability of lung function in severe Alpha-1 Antitrypsin Deficiency
Human Heredity
Alpha-1 antitrypsin aerosolized augmentation abrogates neutrophil elastase-induced expression of Cathepsin B and matrix metalloprotease 2 in vivo and in vitro
Thorax
Alpha-1 Antitrypsin Deficiency: a conformational disease associated with lung and liver manifestations
Journal of Inherited Metabolic Disease
IL10 polymorphisms are associated with airflow obstruction in severe Alpha-1 Antitrypsin Deficiency
American Journal of Respiratory Cell and Molecular Biology
Alpha-1 Antitrypsin Deficiency: current concepts
Lung
Determinants of airflow obstruction in severe Alpha-1 Antitrypsin Deficiency
Thorax
Alpha-1 Antitrypsin Deficiency and computed tomography findings
Journal of Computer Assisted Tomography
Z alpha-1 antitrypsin polymerizes in the lung and acts as a neutrophil chemoattractant
CHEST
Anti-proteinase 3 antibody activation of neutrophils can be inhibited by alpha-1 antitrypsin
American Journal of Respiratory Cell and Molecular Biology
Alpha-1 Antitrypsin Deficiency: biological answers to clinical questions
The American Journal of the Medical Sciences
Alpha-1 Antitrypsin Deficiency
Irish Medical Journal
Merz, Jon
Ethical issues in identifying and recruiting participants for familial genetic research
American Journal of Medical Genetics
Protecting subjects' interest in genetics research
American Journal of Human Genetics
Miki, Toshio
Quantitative comparison of stem cell marker-positive cells in fetal and term human amnion
Journal of Reproductive Immunology
Production of hepatocyte-like cells from human amnion
Methods in Molecular Biology
Isolation of amniotic epithelial stem cells
Current Protocols in Stem Cell Biology
Hepatocyte transplantation: clinical experience and potential for future use
Cell Transplantation
Miravitlles, Marc
Augmentation therapy for emphysema due to Alpha-1 Antitrypsin Deficiency
Therapeutic Advances in Respiratory Diseases
Alpha-1 Antitrypsin Deficiency: diagnosis and treatment
Journal de Brasileira de Pneumologia
Development and results of the Spanish registry of patients with Alpha-1 Antitrypsin Deficiency
International Journal of Chronic Obstructive Pulmonary Disease
Guidelines for the diagnosis and management of Alpha-1 Antitrypsin Deficiency
Archivos de Bronchoneumologica
Alpha-1 Antitrypsin Deficiency: situation in Spain and development of a screening program
Archivos de Bronchoneumologica
Emphysema due to Alpha-1 Antitrypsin Deficiency: familial study of the YBarcelona variant
CHEST
Rapid screening for Alpha-1 Antitrypsin Deficiency in patients with chronic obstructive pulmonary disease using dried blood specimens
American Journal of Respiratory and Critical Care Medicine
Mullins, Daniel
Major components of the direct medical costs of Alpha-1 Antitrypsin Deficiency
CHEST
Olson, Eric L
Genetic modifiers of liver disease in cystic fibrosis
Journal of the American Medical Association
Perlmutter, David
Regulator of G signaling 16 is a marker for the distinct endoplasmic reticulum stress state associated with aggregated mutant alpha-1 antitrypsin Z in the classical form of Alpha-1 Antitrypsin Deficiency
Journal of Biological Chemistry
ADD66, a gene involved in the endoplasmic reticulum-associated degradation of alpha-1 antitrypsin Z in yeast, facilitates proteasome activity and assembly
Molecular Biology of the Cell
Molecular pathogenesis of Alpha-1 Antitrypsin Deficiency associated liver disease: a meeting review
Hepatology
The role of autophagy in Alpha-1 Antitrypsin Deficiency: a specific cellular response in genetic diseases associated with aggregation-prone proteins
Autophagy
Pathogenesis of chronic liver injury and hapatocellular carcinoma in Alpha-1 Antitrypsin Deficiency
Pediatric Research
Intracellular inclusions containing mutant alpha-1 antitrypsin Z are propagated in the absence of autophagic activity
Journal of Biological Chemistry
Accumulation of mutant alpha-1 antitrypsin Z in the endoplasmic reticulum activates caspases-4 and 12, NFkappaB, and BAP31 but not the unfolded protein response
Journal of Biologic Chemistry
Alpha-1 Antitrypsin Deficiency: a new paradigm for hepatocellular carcinoma in genetic liver disease
Hepatology
Grp78, Grp94, and Grp170 interact with alpha-1 antitrypsin mutants that are retained in the endoplasmic reticulum
American Journal of Gastrointestinal and Liver Physiology
Alpha-1 Antitrypsin Deficiency: diagnosis and treatment
Clinical Liver Disease
Analyses of hepatocellular proliferation in a mouse model of Alpha-1 Antitrypsin Deficiency
Hepatology
Alpha-1 Antitrypsin Deficiency: liver disease associated with retention of a mutant secretory glycoprotein in the endoplasmic reticulum
Methods in Molecular Biology
Liver Injury in Alpha-1 Antitrypsin Deficiency: an aggregated protein induces mitochondrial injury
Journal of Clinical Investigation
Chemical chaperones: a pharmacological strategy for disorders of protein folding and trafficking
Pediatric Research
The cellular response to aggregated proteins associated with human disease
Journal of Clinical Investigation
Fasting in alpha-1 antitrypsin deficient liver: constitutive activation of autophagy
American Journal of Gastrointestinal and Liver Physiology
Cell-specific involvement of HNF-1 beta in alpha-1 antitrypsin gene expression in human respiratory epithelial cells
American Journal of Lung Cellular and Molecular Physiology
The proteasome participates in degradation of mutant alpha-1 antitrypsin Z in the endoplasmic reticulum of hepatoma-derived hepatocytes
Journal of Biological Chemistry
A naturally occurring nonpolymerogenic mutant of alpha-1 antitrypsin characterized by prolonged retention in the endoplasmic reticulum
Journal of Biological Chemistry
Liver injury in Alpha-1 Antitrypsin Deficiency
Clinical Liver Disease
Alpha-1 Antitrypsin Deficiency
Current Treatment Options in Gastroenterology
Retention of mutant alpha-1 antitrypsin Z in endoplasmic reticulum is associated with an autophagic response
American Journal of Gastrointestinal and Liver Physiology
Alpha-1 Antitrypsin Deficiency associated liver disease progresses slowly in some children
Journal of Pediatric Gastroenterology and Nutrition
Circulating serpin tumor markers SCCA1 and SCCA2 are not actively secreted but reside in the cytosol of squamous carcinoma cells
International Journal of Cancer
Chemical chaperones mediate increased secretion of mutant alpha-1 antitrypsin Z: A potential pharmacological strategy for prevention of liver injury and emphysema in Alpha-1 Antitrypsin Deficiency
Proceedings of the National Academy of Sciences
Role of ubiquitin in proteasomal degradation of mutant alpha-1 antitrypsin Z in the endoplasmic reticulum
American Journal of Gastrointestinal and Liver Physiology
Glucosidase and mannosidase inhibitors mediate increased secretion of mutant alpha-1 antitrypsin Z
Journal of Biological Chemistry
Misfolded proteins in the endoplasmic reticulum
Laboratory Investigation
Regulation of alpha-1 antitrypsin gene expression in human intestinal epithelial cell line caco-2 by HNF-1 alpha and HNF-4
American Journal of Physiology
Petrache, Irina
Safety and efficacy of alpha-1 antitrypsin augmentation therapy in the treatment of patients with Alpha-1 Antitrypsin Deficiency
Biologics
Mechanisms of alpha-1 antitrypsin endocytosis by lung endothelium
FASEB
CFTR regulation of intracellular pH and ceramides is required for lung endothelial cell apoptosis
American Journal of Respiratory Cell and Molecular Biology
Spinal ceramide modulates the development of morphine antiociceptive tolerance via peroxynitrite-mediated nitroxidative stress and neuroimmune activation
Journal of Pharmacology Experimental Therapy
Targeted induction of lung endothelial cell apoptosis causes emphysema-like changes in the mouse
Journal of Biological Chemistry
Alpha-1 antitrypsin inhibits caspase-3 activity, preventing lung endothelial cell apoptosis
American Journal of Pathology
A novel antiapoptotic role for alpha-1 antitrypsin in the prevention of pulmonary emphysema
American Journal of Respiratory Critical Care Medicine
Schiff, Eugene
Does the Heterozygous State of Alpha-1 Antitrypsin Deficiency Have a Role in Chronic Liver Diseases? Interim Results of a Large Case-Control Study
Journal of Pediatric Gastroenterology and Nutrition
Shaker, Saher
Quantitative assessment of emphysema distribution in smokers and patients with Alpha-1 Antitrypsin Deficiency
Respiratory Medicine
Shao, Matt
Neutrophil elastase induces MUC5AC mucin production in human airway epithelial cells via a cascade involving protein kinase C, reactive oxygen species and TNF-alpha-converting enzyme
Journal of Immunology
Sharp, Richard
Responsible patient advocacy: perspectives from the Alpha-1 Foundation
American Journal of Medical Genetics
Environmental, occupational and genetic risk factors for Alpha-1 Antitrypsin Deficiency
Environmental Health Perspectives
Shifren, Adrian
Elastin protein levels are a vital modifier affecting normal lung development and susceptibility to asthma
American Journal of Lung Cellular and Molecular Physiology
Sifers, Richard
Single nucleotide polymorphism-mediated translational suppression of endoplasmic reticulum mannosidase 1 modifies the onset of end-stage liver disease in Alpha-1 Antitrypsin Deficiency
Hepatology
The mammalian UPR boosts glycoprotein ERAD by suppressing the proteolytic downregulation of ER mannosidase 1
Journal of Cell Science
Small molecules block the polymerization of Z alpha-1 antitrypsin and increase clearance of intracellular aggregates
Journal of Medicinal Chemistry
Human endoplasmic reticulum mannosidase 1 is subject to regulated proteolysis
Journal of Biological Chemistry
Alpha-1 antitrypsin as a model to assess glycan function in endoplasmic reticulum
Methods
Human EDEM2, a novel homolog of family 47 glycosidases, is involved in ER-associated degradation of glycoproteins
Glycobiology
Elucidation of the molecular logic by which misfolded alpha-1 antitrypsin is preferentially selected for degradation
Proceedings of the National Academy of Sciences
Organizational diversity among distinct glycoprotein endoplasmic reticulum-associated degradation programs
Molecular Biology of the Cell
Spencer, Terry
Sustained transgene expression despite T lymphocyte responses in a clinical trial of rAAV1-AAT gene therapy
Proceedings of the National Academy of Sciences
Alpha-1 antitrypsin protects beta cells from apoptosis
Diabetes
Phase 1 trial of intramuscular injection of a recombinant adeno-associated virus serotype 2 alpha-1 antitrypsin (AAT) vvector in AAT-deficient adults
Human Gene Therapy
Antibody response to aerosolized transgenic human alpha-1 antitrypsin
New England
Journal of Medicine
Role of human neutrophil peptides in lung inflammation associated with Alpha-1 Antitrypsin Deficiency
American Journal of Lung Cellular and Molecular Physiology
Song, Sihong
Alpha-1 antitrypsin protects beta cells from apoptosis
Diabetes
Phase 1 trial of intramuscular injection of a recombinant adeno-associated virus serotype 2 alpha-1 antitrypsin (AAT) vvector in AAT-deficient adults
Human Gene Therapy
Alpha-1 antitrypsin gene therapy modulates cellular immunity and efficiently prevents type 1 diabetes in nonobese diabetic mice
Human Gene Therapy
Therapeutic level of functional human alpha-1 antitrypsin (hAAT) secreted from murine muscle transduced by adeno-associated virus (rAAV1) vector
Journal of Gene Medicine
A novel antiapoptotic role for alpha-1 antitrypsin in the prevention of pulmonary emphysema
American Journal of Respiratory and Critical Care Medicine
Efficient hepatic delivery and expression from a recombinant adeno-associated virus 8 pseudotyped alpha-1 antitrypsin vector
Molecular Therapy
Enhancing rAAV vector expression in the lung
Journal of Gene Medicine
Intramuscular administration of recombinant adeno-associated virus 2 alpha-1 antitrypsin (rAAV-SERPINA1) vectors in a nonhuman primate model: safety and immunologic aspects
Molecular Therapy
Improved method of recombinant AAV2 delivery for systemic targeted gene therapy
Molecular Therapy
Steffen, Martin
Possible role of the phagocytic protinases, cathepsin B and elastase, in orthotopic liver transplantation
Transplant Proceedings
Stoller, James
Physician alert suggesting Alpha-1 Antitrypsin Deficiency testing in pulmonary function test results
COPD
New findings in PiZZ Alpha-1 Antitrypsin Deficiency related panniculitis. Demonstration of skin polymers and high dosing requirements of intravenous augmentation therapy
Dermatology
Detection of Alpha-1 Antitrypsin Deficiency: a review
Respiratory Medicine
Heritability of lung function in severe Alpha-1 Antitrypsin Deficiency
Human Heredity
Alpha-1 antitrypsin augmentation therapy for PiMZ heterozygotes: a cautionary note
CHEST
Augmentation therapy in Alpha-1 Antitrypsin Deficiency
Expert Opinion on Biological Therapy
Primary care diagnosis of Alpha-1 Antitrypsin Deficiency: issues and opportunities
Cleveland Clinic Journal of Medicine
Risk factors for symptom onset in PiZ Alpha-1 Antitrypsin Deficiency
International Journal of Chronic Obstructive Pulmonary Disease
Therapy for Alpha-1 Antitrypsin Deficiency: pharmacology and clinical recomendations
BioDrugs
IL10 polymorphisms are associated with airflow obstruction in severe Alpha-1 Antitrypsin Deficiency
American Journal of Respiratory Cell and Molecular Biology
Determinants of airflow obstruction in severe Alpha-1 Antitrypsin Deficiency
Thorax
Lung volume reduction surgery in patients with emphysema and Alpha-1 Antitrypsin Deficiency
Annals of Thoracic Surgery
Cost-effectiveness of population screening for Alpha-1 Antitrypsin Deficiency: a decision analysis
COPD
A review of Alpha-1 Antitrypsin Deficiency
COPD
A review of the Alpha-1 Foundation: its formation, impact, and critical success factors
Respiratory Care
Delay in diagnosis of Alpha-1 Antitrypsin Deficiency: a continuing problem
CHEST
Results of a survey of patients with Alpha-1 Antitrypsin Deficiency
Respiration
A review of Alpha-1 Antitrypsin Deficiency
Seminars in Respiratory and Critical Care Medicine
Alpha-1 Antitrypsin Deficiency
Lancet
Mortality in individuals with severe Alpha-1 Antitrypsin Deficiency: findings from the National Heart, Lung, and Blood Institute Registry
CHEST
The bronchopulmonary pathology of Alpha-1 Antitrypsin Deficiency: findings of the Death Review Committee on the national registry for individuals with Severe Deficiency of alpha-1 antitrypsin
Human Pathology
High dose intravenous AAT and plasma neutrophil derived fibrinogen fragments
Thorax
Augmentation therapy for Alpha-1 Antitrypsin Deficiency
Drugs
Alpha-1 Antitrypsin Deficiency: intravenous augmentation therapy: current understanding
Thorax
Alpha-1 Antitrypsin Deficiency
Thorax
Key current clinical issues in Alpha-1 Antitrypsin Deficiency
Respiratory Care
Alpha-1 Antitrypsin Deficiency: an under-recognized but important issue for respiratory therapists
Respiratory Care
Major components of the direct medical costs of Alpha-1 Antitrypsin Deficiency
CHEST
Augmentation therapy with Alpha-1 Antitrypsin Deficiency: patterns of use and adverse events
CHEST
Asthma features in severe Alpha-1 Antitrypsin Deficiency: experience of the National Heart, Lung, and Blood Institute Registry
CHEST
Cost-effectiveness analysis of augmentation therapy for severe Alpha-1 Antitrypsin Deficiency
American Journal of Respiratory and Critical Care Medicine
Emphysema in nonsmokers: Alpha-1 Antitrypsin Deficiency and other causes
Cleveland Clinic Journal of Medicine
Low levels of nitric oxide and carbon monoxide in Alpha-1 Antitrypsin Deficiency
Journal of Applied Physiology
Biochemical efficacy and safety of a new pooled human plasma alpha-1 antitrypsin, Respitin
CHEST
The direct medical costs of Alpha-1 Antitrypsin Deficiency
CHEST
Formation and current results of a patient-organized registry for Alpha-1 Antitrypsin Deficiency
CHEST
Occupational exposure risks in individuals with PiZ Alpha-1 Antitrypsin Deficiency
American Journal of Respiratory and Critical Care Medicine
Teckman, Jeffrey
Alpha-1 antitrypsin mutant Z protein content in individual hepatocytes correlates with call death in a mouse model
Hepatology
Alpha-1 antitrypsin deficiency in childhood
Seminar in Liver Disease
In vivo post-transcriptional gene silencing of alpha-1 antitrypsin by adeno-associated virus vectors expressing siRNA
Laboratory Investigation
Indomethacin increases liver damage in a murine model of liver injury from alpha-1 antitrypsin deficiency
Hepatology
Alpha-1 Antitrypsin Deficiency: diagnosis, pathophysiology, and management
Current Gastroenterology Reports
Quantitative isolation of alpha-1 antitrypsin mutant Z protein polymers from human an mouse livers and the effect of heat
Hepatology
Lack of effect of oral 4-phenylbutyrate on serum alpha-1 antitrypsin in patients with alpha-1 antitrypsin deficiency: a preliminary study
Journal of Pediatric Gastroenterology and Nutrition
Analyses of hepatocellular proliferation in a mouse model of Alpha-1 Antitrypsin Deficiency
Hepatology
Mitochondrial autophagy and injury in the liver in Alpha-1 Antitrypsin Deficiency
American Journal of Physiology - Gastrointestinal and Liver Physiology
Fasting in alpha-1 antitrypsin deficient liver: constitutive [correction of consultative]
American Journal of Physiology - Gastrointestinal and Liver Physiology
The proteasome participants in degradation of mutant alpha-1 antitrypsin Z in the enoplasmic reticulum of hepatoma-derived hepatocytes
Journal of Biological Chemistry
A naturally occurring nonpolymerogenic mutant of alpha-1 antitrypsin characterized by prolonged retention in the endoplasmic reticulum
Journal of Biological Chemistry
Retention of mutant alpha-1 antitrypsin Z in endoplasmic reticulum is associated with an autophagic response
American Journal of Physiology - Gastrointestinal and Liver Physiology
Role of ubiquitin in proteasomal degradation of mutant alpha-1 antitrypsin Z in the endoplasmic reticulum
American Journal of Physiology - Gastrointestinal and Liver Physiology
Tuder, Rubin
Alpha-1 antitrypsin inhibits caspase-3 activity, preventing lung endothelial cell apoptosis
American Journal of Pathology
A novel antiapoptic role for alpha-1 antitrypsin in the prevention of pulmonary emphysema
American Journal of Respiratory and Critical Care Medicine
Oxidative stress and apoptosis interact and cause emphysema due to vascular endothelial growth factor receptor blockade
American Journal of Respiratory Cell and Molecular Biology
Turino, Gerry
Alpha-1 Antitrypsin Deficieny: Lessons from longetivity
CHEST
Prevalence of Alpha-1 Antitrypsin Deficiency in poorly controlled asthma—Results from the ALA-ACRC low dose theophylline trial
Journal of Asthma
Determinants of airflow obstruction in severe alpha-1-antitrypsin deficiency
Thorax
Aerosolized hyaluronan limits airspace enlargement in a mouse model of cigarette smoke induced pulmonary emphysema
Experimental Lung Research
The detection and quantitation of free desmosine and isodesmosine in human urine and their peptide-bound forms in sputum
Proceedings of the National Academy of Sciences
Asthma features in sever Alpha-1 Antitrypsin Deficiency: experience of the National Heart, Lung, and Blood Institute Registry
CHEST
Feasibility of a clinical trial of augmentation therapy for Alpha-1 Antitrypsin Deficiency. The Alpha-1 Antitrypsin Deficiency Registry Study Group
American Journal of Respiratory and Critical Care Medicine
Vedal, Sverre
Risk factors for symptom onset in PI*Z Alpha-1 Antitrypsin Deficiency
International Journal of Chronic Obstructive Pulmonary Disease
Verfaillie, Catherine
Sequential exposure to cytokines reflecting embryogenesis: the key for in vitro differentiation of adult bone marrow stem cells into functional hepatocyte-like cells
Toxicology Science
Wilson, Andrew
Sustained expression of alpha-1 antitrypsin after transplantation of manipulated hematopoietic stem cells
American Journal of Respiratory Cell and Molecular Biology
Wood, Alice
Mortality in Alpha-1 Antitrypsin Deficiency in the United Kingdom
Respiratory Medicine
Phenotypic differences in alpha-1 antitrypsin-deficient sibling pairs may relate to genetic variation
COPD
Matrix metalloprotease polymorphisms are associated with gas transfer in Alpha-1 Antitrypsin Deficiency
Therapeutic Advances in Respiratory Disease
The TNF alpha gene relates to clinical phenotype in Alpha-1 Antitrypsin Deficiency
Respiratory Research
The genetics of chronic obstructive pulmonary disease
Respiratory Research
Yarborough, Mark
Responsible patient advocacy: perspectives from the Alpha-1 Foundation
American Journal of Medical Genetics Part A
Yu, Hong
Inhibition of mouse hepatocyte apoptosis by anti-caspase-12 small interfering RNA
Chinese Journal of Hepatology
Zern, Mark
Cell transplantation to replace whole liver transplantation
Gastroenterology
Differentiation in enrichment of hepatocyte-like cells from human embryonic stem cells in vitro and in vivo
Stem Cells
Recent progress of pathogenesis and treatment in Alpha-1 Antitrypsin Deficiency
Japanese Journal of Clinical Medicine
Ribozyme-mediated specific gene replacement of the alpha-1 antitrypsin gene in human hepatoma cells
Journal of Hepatology
A novel SV40-based vector successfully transduces and expresses an alpha-1 antitrypsin ribozyme in a human hepatoma-derived cell line
Gene Therapy
Zhou, Aiwu
Targeting a surface cavity of alpha-1 antitrypsin to prevent conformational disease
Journal of Biological Chemistry
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