Related references
Note: Only part of the references are listed.Neutrophilic panniculitis associated with alpha-1-antitrypsin deficiency: an update
I. Blanco et al.
BRITISH JOURNAL OF DERMATOLOGY (2016)
Polymers of Z α1-antitrypsin are secreted in cell models of disease
Annamaria Fra et al.
EUROPEAN RESPIRATORY JOURNAL (2016)
Alpha-1-antitrypsin (SERPINA1) mutation spectrum: Three novel variants and haplotype characterization of rare deficiency alleles identified in Portugal
Deolinda Silva et al.
RESPIRATORY MEDICINE (2016)
Unusual Acute Sequelae of α1-Antitrypsin Deficiency A Myriad of Symptoms With One Common Cure
Alessandro N. Franciosi et al.
CHEST (2015)
The United States Alpha-1 Foundation Research Registry: Genesis, Impact and Future
Charlie Strange et al.
COPD-JOURNAL OF CHRONIC OBSTRUCTIVE PULMONARY DISEASE (2015)
The Impact of Smoke Exposure on the Clinical Phenotype of Alpha-1 Antitrypsin Deficiency in Ireland: Exploiting a National Registry to Understand a Rare Disease
M. Emmet O'Brien et al.
COPD-JOURNAL OF CHRONIC OBSTRUCTIVE PULMONARY DISEASE (2015)
The Clinical Profile of Subjects Included in the Swedish National Register on Individuals with Severe Alpha 1-Antitrypsin deficiency
Eeva Piitulainen et al.
COPD-JOURNAL OF CHRONIC OBSTRUCTIVE PULMONARY DISEASE (2015)
The National Alpha-1 Antitrypsin Deficiency Registry in Poland
Joanna Chorostowska-Wynimko et al.
COPD-JOURNAL OF CHRONIC OBSTRUCTIVE PULMONARY DISEASE (2015)
Induced pluripotent stem cells model personalized variations in liver disease resulting from 1-antitrypsin deficiency
Edgar N. Tafaleng et al.
HEPATOLOGY (2015)
The BLT1 Inhibitory Function of α-1 Antitrypsin Augmentation Therapy Disrupts Leukotriene B4 Neutrophil Signaling
Ciara A. O'Dwyer et al.
JOURNAL OF IMMUNOLOGY (2015)
Alternative transcripts of the SERPINA1 gene in alpha-1 antitrypsin deficiency
Nerea Matamala et al.
JOURNAL OF TRANSLATIONAL MEDICINE (2015)
Intravenous augmentation treatment and lung density in severe α1 antitrypsin deficiency (RAPID): a randomised, double-blind, placebo-controlled trial
Kenneth R. Chapman et al.
LANCET (2015)
An integrative approach combining ion mobility mass spectrometry, X-ray crystallography, and nuclear magnetic resonance spectroscopy to study the conformational dynamics of (1)-antitrypsin upon ligand binding
Mun Peak Nyon et al.
PROTEIN SCIENCE (2015)
Emergence of a Stage-Dependent Human Liver Disease Signature with Directed Differentiation of Alpha-1 Antitrypsin-Deficient iPS Cells
Andrew A. Wilson et al.
STEM CELL REPORTS (2015)
Alpha-1 proteinase inhibitors for the treatment of alpha-1 antitrypsin deficiency: safety, tolerability, and patient outcomes
Sanjay H. Chotirmall et al.
Therapeutics and Clinical Risk Management (2015)
The efficacy and safety of inhaled human α-1 antitrypsin in people with α-1 antitrypsin deficiency-related emphysema
Alessandro N. Franciosi et al.
EXPERT REVIEW OF RESPIRATORY MEDICINE (2015)
Function of monocytes and monocyte-derived macrophages in α1-antitrypsin deficiency
Emily F. A. van't Wout et al.
EUROPEAN RESPIRATORY JOURNAL (2015)
Current status of gene therapy for α-1 antitrypsin deficiency
Heather S. Loring et al.
EXPERT OPINION ON BIOLOGICAL THERAPY (2015)
Alpha-1 antitrypsin and liver disease: mechanisms of injury and novel interventions
Jeffrey H. Teckman et al.
EXPERT REVIEW OF GASTROENTEROLOGY & HEPATOLOGY (2015)
The Swedish α1-Antitrypsin Screening Study: Health Status and Lung and Liver Function at Age 34
Hanan A. Tanash et al.
ANNALS OF THE AMERICAN THORACIC SOCIETY (2015)
Diagnosing α1-antitrypsin deficiency: how to improve the current algorithm
Noel G. McElvaney
EUROPEAN RESPIRATORY REVIEW (2015)
Cellular Mechanisms of Endoplasmic Reticulum Stress Signaling in Health and Disease. 2. Protein misfolding and ER stress
Joseph E. Chambers et al.
AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY (2014)
α1-Antitrypsin Activates Protein Phosphatase 2A to Counter Lung Inflammatory Responses
Patrick Geraghty et al.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2014)
Z α1-Antitrypsin Confers a Proinflammatory Phenotype That Contributes to Chronic Obstructive Pulmonary Disease
Samuel Alam et al.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2014)
Clarification of the Risk of Chronic Obstructive Pulmonary Disease in α1-Antitrypsin Deficiency PiMZ Heterozygotes
Kevin Molloy et al.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2014)
Altered native stability is the dominant basis for susceptibility of α1-antitrypsin mutants to polymerization
James A. Irving et al.
BIOCHEMICAL JOURNAL (2014)
The Shapes of Z-α1-Antitrypsin Polymers in Solution Support the C-Terminal Domain-Swap Mechanism of Polymerization
Manja A. Behrens et al.
BIOPHYSICAL JOURNAL (2014)
Circulating polymers in alpha(1)-antitrypsin deficiency
Lu Tan et al.
EUROPEAN RESPIRATORY JOURNAL (2014)
Therapeutic targeting of misfolding and conformational change in α1-antitrypsin deficiency
Mun Peak Nyon et al.
FUTURE MEDICINAL CHEMISTRY (2014)
Increased ERK signalling promotes inflammatory signalling in primary airway epithelial cells expressing Zα1-antitrypsin
Emily F. A. van 't Wout et al.
HUMAN MOLECULAR GENETICS (2014)
Antisense oligonucleotide treatment ameliorates alpha-1 antitrypsin-related liver disease in mice
Shuling Guo et al.
JOURNAL OF CLINICAL INVESTIGATION (2014)
Alpha-1 Antitrypsin Augmentation Therapy Corrects Accelerated Neutrophil Apoptosis in Deficient Individuals
Killian Hurley et al.
JOURNAL OF IMMUNOLOGY (2014)
Interactions between SERPINA1 PiMZ genotype, occupational exposure and lung function decline
A. J. Mehta et al.
OCCUPATIONAL AND ENVIRONMENTAL MEDICINE (2014)
Identification and characterisation of eight novel SERPINA1 Null mutations
Ilaria Ferrarotti et al.
ORPHANET JOURNAL OF RARE DISEASES (2014)
The Circulating Proteinase Inhibitor α-1 Antitrypsin Regulates Neutrophil Degranulation and Autoimmunity
David A. Bergin et al.
SCIENCE TRANSLATIONAL MEDICINE (2014)
The molecular and cellular pathology of α1-antitrypsin deficiency
Bibek Gooptu et al.
TRENDS IN MOLECULAR MEDICINE (2014)
Modulation of the Maladaptive Stress Response to Manage Diseases of Protein Folding
Daniela Martino Roth et al.
PLOS BIOLOGY (2014)
Phase I/II Study of Intrapleural Administration of a Serotype rh.10 Replication-Deficient Adeno-Associated Virus Gene Transfer Vector Expressing the Human α1-Antitrypsin cDNA to Individuals with α1-Antitrypsin Deficiency
Maria J. Chiuchiolo et al.
HUMAN GENE THERAPY CLINICAL DEVELOPMENT (2014)
The Challenge of Detecting Alpha-1 Antitrypsin Deficiency
James K. Stoller et al.
COPD-JOURNAL OF CHRONIC OBSTRUCTIVE PULMONARY DISEASE (2013)
Differences in Adjustment between Individuals with Alpha-1 Antitrypsin Deficiency (AATD)-Associated COPD and Non-AATD COPD
Kristen E. Holm et al.
COPD-JOURNAL OF CHRONIC OBSTRUCTIVE PULMONARY DISEASE (2013)
The Electrophoretic α1-Globulin Pattern of Serum in α1-Antitrypsin Deficiency
Carl-Bertil Laurell et al.
COPD-JOURNAL OF CHRONIC OBSTRUCTIVE PULMONARY DISEASE (2013)
Endoplasmic Reticulum Polymers Impair Luminal Protein Mobility and Sensitize to Cellular Stress in Alpha1-Antitrypsin Deficiency
Adriana Ordonez et al.
HEPATOLOGY (2013)
Human Treg responses allow sustained recombinant adeno-associated virus-mediated transgene expression
Christian Mueller et al.
JOURNAL OF CLINICAL INVESTIGATION (2013)
Endoplasmic reticulum dysfunction in neurological disease
Benoit D. Roussel et al.
LANCET NEUROLOGY (2013)
Alphai-antitrypsin deficiency - Diagnostic testing and disease awareness in Germany and Italy
Timm Greulich et al.
RESPIRATORY MEDICINE (2013)
Causal and Synthetic Associations of Variants in the SERPINA Gene Cluster with Alpha1-antitrypsin Serum Levels
Gian Andri Thun et al.
PLOS GENETICS (2013)
Diagnosis and Management of Patients With α1-Antitrypsin (A1AT) Deficiency
David R. Nelson et al.
CLINICAL GASTROENTEROLOGY AND HEPATOLOGY (2012)
Histone Deacetylase Inhibitor (HDACi) Suberoylanilide Hydroxamic Acid (SAHA)-mediated Correction of α1-Antitrypsin Deficiency
Marion Bouchecareilh et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2012)
In Silico Assessment of Potential Druggable Pockets on the Surface of α1-Antitrypsin Conformers
Anathe O. M. Patschull et al.
PLOS ONE (2012)
Association of IREB2 and CHRNA3 polymorphisms with airflow obstruction in severe alpha-1 antitrypsin deficiency
Woo Jin Kim et al.
RESPIRATORY RESEARCH (2012)
Alpha-1-antitrypsin augmentation therapy in deficient individuals enrolled in the Alpha-1 Foundation DNA and Tissue Bank
Adriano Tonelli
International Journal of Chronic Obstructive Pulmonary Disease (2012)
Impact of a Clinical Decision Support System in an Electronic Health Record to Enhance Detection of α1-Antitrypsin Deficiency
Anil Jain et al.
CHEST (2011)
Long-term outcome in 42 pediatric liver transplant patients with alpha 1-antitrypsin deficiency: a single-center experience
Michael G. Hughes et al.
CLINICAL TRANSPLANTATION (2011)
Molecular basis of α1-antitrypsin deficiency revealed by the structure of a domain-swapped trimer
Masayuki Yamasaki et al.
EMBO REPORTS (2011)
Preclinical Evaluation of a Recombinant Adeno-Associated Virus Vector Expressing Human Alpha-1 Antitrypsin Made Using a Recombinant Herpes Simplex Virus Production Method
Jeffrey D. Chulay et al.
HUMAN GENE THERAPY (2011)
Phase 2 Clinical Trial of a Recombinant Adeno-Associated Viral Vector Expressing α1-Antitrypsin: Interim Results
Terence R. Flotte et al.
HUMAN GENE THERAPY (2011)
Targeted gene correction of α1-antitrypsin deficiency in induced pluripotent stem cells
Kosuke Yusa et al.
NATURE (2011)
Dynamic local unfolding in the serpin α-1 antitrypsin provides a mechanism for loop insertion and polymerization
Beena Krishnan et al.
NATURE STRUCTURAL & MOLECULAR BIOLOGY (2011)
Living Related Donor Liver Transplantation in Children
A. C. A. Tannuri et al.
TRANSPLANTATION PROCEEDINGS (2011)
The role of proteases, endoplasmic reticulum stress and SERPINA1 heterozygosity in lung disease and α-1 anti-trypsin deficiency
Catherine M. Greene et al.
EXPERT REVIEW OF RESPIRATORY MEDICINE (2011)
The prevalence of alpha-1 antitrypsin deficiency in Ireland
Tomas P. Carroll et al.
RESPIRATORY RESEARCH (2011)
Rapamycin reduces intrahepatic alpha-1-antitrypsin mutant Z protein polymers and liver injury in a mouse model
Shalesh Kaushal et al.
EXPERIMENTAL BIOLOGY AND MEDICINE (2010)
A Novel Monoclonal Antibody to Characterize Pathogenic Polymers in Liver Disease Associated with α1-Antitrypsin Deficiency
Elena Miranda et al.
HEPATOLOGY (2010)
Modeling inherited metabolic disorders of the liver using human induced pluripotent stem cells
S. Tamir Rashid et al.
JOURNAL OF CLINICAL INVESTIGATION (2010)
α-1 Antitrypsin regulates human neutrophil chemotaxis induced by soluble immune complexes and IL-8
David A. Bergin et al.
JOURNAL OF CLINICAL INVESTIGATION (2010)
Evidence for Unfolded Protein Response Activation in Monocytes from Individuals with α-1 Antitrypsin Deficiency
Tomas P. Carroll et al.
JOURNAL OF IMMUNOLOGY (2010)
Alpha(sub 1)-Antitrypsin Deficiency and Autophagy.
Stefan J. Marciniak et al.
NEW ENGLAND JOURNAL OF MEDICINE (2010)
Outdoor air pollution is associated with rapid decline of lung function in α-1-antitrypsin deficiency
Alice M. Wood et al.
OCCUPATIONAL AND ENVIRONMENTAL MEDICINE (2010)
Defining the mechanism of polymerization in the serpinopathies
Ugo I. Ekeowa et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2010)
An Autophagy-Enhancing Drug Promotes Degradation of Mutant α1-Antitrypsin Z and Reduces Hepatic Fibrosis
Tunda Hidvegi et al.
SCIENCE (2010)
Establishment of lentiviral-vector-mediated model of human alpha-1 antitrypsin delivery into hepatocyte-like cells differentiated from mesenchymal stem cells
Mahboobe Ghaedi et al.
TISSUE & CELL (2010)
Clinical Characteristics of Subjects With Symptoms of α1-Antitrypsin Deficiency Older Than 60 Years
Michael A. Campos et al.
CHEST (2009)
Effects of a Disease Management Program in Individuals with Alpha-1 Antitrypsin Deficiency
Michael A. Campos et al.
COPD-JOURNAL OF CHRONIC OBSTRUCTIVE PULMONARY DISEASE (2009)
Outdoor air pollution is associated with disease severity in α1-antitrypsin deficiency
A. M. Wood et al.
EUROPEAN RESPIRATORY JOURNAL (2009)
Exploring the role of CT densitometry: a randomised study of augmentation therapy in α1-antitrypsin deficiency
A. Dirksen et al.
EUROPEAN RESPIRATORY JOURNAL (2009)
Neuroserpin Polymers Activate NF-κB by a Calcium Signaling Pathway That Is Independent of the Unfolded Protein Response
Mark J. Davies et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2009)
Endoplasmic Reticulum-associated Degradation (ERAD) and Autophagy Cooperate to Degrade Polymerogenic Mutant Serpins
Heike Kroeger et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2009)
Small-molecule peptides inhibit Z α1-antitrypsin polymerization
Yi-Pin Chang et al.
JOURNAL OF CELLULAR AND MOLECULAR MEDICINE (2009)
Decreased Levels of Secretory Leucoprotease Inhibitor in the Pseudomonas-Infected Cystic Fibrosis Lung Are Due to Neutrophil Elastase Degradation
Sinead Weldon et al.
JOURNAL OF IMMUNOLOGY (2009)
Crystallographic and Cellular Characterisation of Two Mechanisms Stabilising the Native Fold of α1-Antitrypsin: Implications for Disease and Drug Design
Bibek Gooptu et al.
JOURNAL OF MOLECULAR BIOLOGY (2009)
Recombinant AAV Serotype and Capsid Mutant Comparison for Pulmonary Gene Transfer of alpha-1-Antitrypsin Using Invasive and Noninvasive Delivery
Rejean Liqun Wang et al.
MOLECULAR THERAPY (2009)
Sustained transgene expression despite T lymphocyte responses in a clinical trial of rAAV1-AAT gene therapy
Mark L. Brantly et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2009)
Matrix metalloprotease polymorphisms are associated with gas transfer in alpha 1 antitrypsin deficiency
Christopher J. McAloon et al.
Therapeutic Advances in Respiratory Disease (2009)
IL10 polymorphisms are associated with airflow obstruction in severe alpha(1)-antitrypsin deficiency
Dawn L. DeMeo et al.
AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY (2008)
Persistent episomal transgene expression in liver following delivery of a scaffold/matrix attachment region containing non-viral vector
O. Argyros et al.
GENE THERAPY (2008)
Elafin, an Elastase-specific Inhibitor, Is Cleaved by Its Cognate Enzyme Neutrophil Elastase in Sputum from Individuals with Cystic Fibrosis
Nicolas Guyot et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2008)
Activation of the Epidermal Growth Factor Receptor (EGFR) by a Novel Metalloprotease Pathway
David A. Bergin et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2008)
Crystal structure of a stable dimer reveals the molecular basis of serpin polymerization
Masayuki Yamasaki et al.
NATURE (2008)
Adapting proteostasis for disease intervention
William E. Balch et al.
SCIENCE (2008)
Clinical course and prognosis of never-smokers with severe alpha-1-antitrypsin deficiency (PiZZ)
H. A. Tanash et al.
THORAX (2008)
The TNFalpha gene relates to clinical phenotype in alpha-I-antitrypsin deficiency
Alice M. Wood et al.
RESPIRATORY RESEARCH (2008)
Cleavage of CXCR1 on neutrophils disables bacterial killing in cystic fibrosis lung disease
Dominik Hartl et al.
NATURE MEDICINE (2007)
Small molecules block the polymerization of Z α1-antitrypsin and increase the clearance of intracellular aggregates
Meera Mallyat et al.
JOURNAL OF MEDICINAL CHEMISTRY (2007)
Alpha-1-antitrypsin mutant Z protein content in individual Hepatocytes correlates with cell death in a mouse model
Douglas Lindblad et al.
HEPATOLOGY (2007)
Determinants of airflow obstruction in severe alpha-1-antitrypsin deficiency
Dawn L. DeMeo et al.
THORAX (2007)
Signal integration in the endoplasmic reticulum unfolded protein response
David Ron et al.
NATURE REVIEWS MOLECULAR CELL BIOLOGY (2007)
Neutrophil elastase up-regulates cathepsin B and matrix metalloprotease-2 expression
Patrick Geraghty et al.
JOURNAL OF IMMUNOLOGY (2007)
Ongoing research in Europe: Alpha One International Registry (AIR) objectives and development
R. A. Stockley et al.
EUROPEAN RESPIRATORY JOURNAL (2007)
α1-antitrypsin inhalation reduces airway inflammation in cystic fibrosis patients
M. Griese et al.
EUROPEAN RESPIRATORY JOURNAL (2007)
Phase I trial of intramuscular injection of a recombinant adeno-associated virus serotype 2 α1-antitrypsin (AAT) vector in AAT-deficient adults
Mark L. Brantly et al.
HUMAN GENE THERAPY (2006)
α-1 Antitrypsin inhibits caspase-3 activity, preventing lung endothelial cell apoptosis
Irina Petrache et al.
AMERICAN JOURNAL OF PATHOLOGY (2006)
Genetic testing of minors for alpha1-antitrypsin deficiency
C Strange et al.
ARCHIVES OF PEDIATRICS & ADOLESCENT MEDICINE (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 alpha-1 proteinase inhibitor (A1PiZ) and another for aggregates of A1PiZ
KB Kruse et al.
MOLECULAR BIOLOGY OF THE CELL (2006)
On the mechanism of sensing unfolded protein in the endoplasmic reticulum
JJ Credle et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2005)
Accumulation of mutant α1-antitrypsin Z in the endoplasmic reticulum activates caspases-4 and -12, NFκB, and BAP31 but not the unfolded protein response
T Hidvegi et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2005)
Neutrophil elastase induces MUC5AC mucin production in human airway epithelial cells via a cascade involving protein kinase C, reactive oxygen species, and TNF-α-converting enzyme
MXG Shao et al.
JOURNAL OF IMMUNOLOGY (2005)
Expression and secretion of alpha1-proteinase inhibitor are regulated by proinflammatory cytokines in human pancreatic islet cells
D Bosco et al.
DIABETOLOGIA (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 et al.
CYTOKINE (2005)
AASLD practice guidelines: Evaluation of the patient for liver transplantation
KF Murray et al.
HEPATOLOGY (2005)
Polymers of Z α1-antitrypsin co-localize with neutrophils in emphysematous alveoli and are chemotactic in vivo
R Mahadeva et al.
AMERICAN JOURNAL OF PATHOLOGY (2005)
Dual oxidase 1-dependent MUC5AC mucin expression in cultured human airway epithelial cells
MXG Shao et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2005)
Activation of endoplasmic reticulum-specific stress responses associated with the conformational disease Z α1-antitrypsin deficiency
MW Lawless et al.
JOURNAL OF IMMUNOLOGY (2004)
Inhibiting polymerization -: New therapeutic strategies for z α1-antitrypsin-related emphysema
H Parfrey et al.
AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY (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)
Genetic testing for Alpha1-antitrypsin deficiency
C Strange et al.
GENETICS IN MEDICINE (2004)
α1-Antitrypsin deficiency • 3:: Clinical manifestations and natural history
M Needham et al.
THORAX (2004)
Predictors of mortality in α1-antitrypsin deficiency
PA Dawkins et al.
THORAX (2003)
American Thoracic Society/European Respiratory Society Statement: Standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2003)
Enhancement of endoplasmic reticulum (ER) degradation of misfolded null Hong Kong α1-antitrypsin by human ER mannosidase I
N Hosokawa et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2003)
Elucidation of the molecular logic by which misfolded α1-antitrypsin is preferentially selected for degradation
Y Wu et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2003)
Organizational diversity among distinct glycoprotein endoplasmic reticulum-associated degradation programs
CM Cabral et al.
MOLECULAR BIOLOGY OF THE CELL (2002)
Polymers of α1-antitrypsin are chemotactic for human neutrophils -: A new paradigm for the pathogenesis of emphysema
JS Parmar et al.
AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY (2002)
Gene regulation of the serine proteinase inhibitors α1-antitrypsin and α1-antichymotrypsin
N Kalsheker et al.
BIOCHEMICAL SOCIETY TRANSACTIONS (2002)
6-mer peptide selectively anneals to a pathogenic serpin conformation and blocks polymerization - Implications for the prevention of Z alpha(1)-antitrypsin-related cirrhosis
R Mahadeva et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2002)
The proteasome participates in degradation of mutant α1-antitrypsin Z in the endoplasmic reticulum of hepatoma-derived hepatocytes
JH Teckman et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2001)
Longitudinal follow-up of patients with α1-protease inhibitor deficiency before and during therapy with IV α1-protease inhibitor
M Wencker et al.
CHEST (2001)
Cleavage of CD14 on human gingival fibroblasts cocultured with activated neutrophils is mediated by human leukocyte elastase resulting in down-regulation of lipopolysaccharide-induced IL-8 production
E Nemoto et al.
JOURNAL OF IMMUNOLOGY (2000)
Augmentation therapy reduces frequency of lung infections in antitrypsin deficiency - A new hypothesis with supporting data
J Lieberman
CHEST (2000)
Structure of a serpin-protease complex shows inhibition by deformation
JA Huntington et al.
NATURE (2000)
Processing by endoplasmic reticulum mannosidases partitions a secretion-impaired glycoprotein into distinct disposal pathways
CM Cabral et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2000)
Occupational exposure risks in individuals with PI*Z α1-antitrypsin deficiency
AS Mayer et al.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2000)
Dynamic interaction of BiP and ER stress transducers in the unfolded-protein response
A Bertolotti et al.
NATURE CELL BIOLOGY (2000)
Differential regulation of gene activity and chromatin structure within the human serpin gene cluster at 14q32.1 in macrophage microcell hybrids
Pierre Rollini et al.
NUCLEIC ACIDS RESEARCH (2000)
Chemical chaperones mediate increased secretion of mutant α1-antitrypsin (α1-AT) Z:: A potential pharmacological strategy for prevention of liver injury and emphysema in α1-AT deficiency
JAJ Burrows et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2000)
Share Paper
