(Q37894093)

24 August 2017

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title

The role of proteases, endoplasmic reticulum stress and SERPINA1 heterozygosity in lung disease and α-1 anti-trypsin deficiency (English)

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24 August 2017

main subject

endoplasmic reticulum

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Catherine M Greene

1

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author name string

Tidi Hassan

series ordinal

2

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24 August 2017

Kevin Molloy

series ordinal

3

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24 August 2017

Noel G McElvaney

series ordinal

4

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24 August 2017

publication date

1 June 2011

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Expert Review of Respiratory Medicine

24 August 2017

published in

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24 August 2017

volume

5

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24 August 2017

issue

3

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24 August 2017

page(s)

395-411

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The mechanism of Z alpha 1-antitrypsin accumulation in the liver

24 August 2017

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Delay in diagnosis of alpha1-antitrypsin deficiency: a continuing problem

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Proteins of the cystic fibrosis respiratory tract. Fragmented immunoglobulin G opsonic antibody causing defective opsonophagocytosis

21 January 2018

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Reduction of ciliary beat frequency in vitro by sputum from patients with bronchiectasis: a serine proteinase effect

21 January 2018

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Decreased levels of secretory leucoprotease inhibitor in the Pseudomonas-infected cystic fibrosis lung are due to neutrophil elastase degradation

21 January 2018

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Elafin, an elastase-specific inhibitor, is cleaved by its cognate enzyme neutrophil elastase in sputum from individuals with cystic fibrosis

21 January 2018

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Pattern of emphysema distribution in alpha1-antitrypsin deficiency influences lung function impairment

21 January 2018

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Correlation between annual change in health status and computer tomography derived lung density in subjects with alpha1-antitrypsin deficiency

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Decline in FEV1 among patients with severe hereditary alpha 1-antitrypsin deficiency type PiZ.

21 January 2018

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IL10 polymorphisms are associated with airflow obstruction in severe alpha1-antitrypsin deficiency.

21 January 2018

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Tumor necrosis factor-{alpha} rs361525 polymorphism is associated with increased local production and downstream inflammation in chronic obstructive pulmonary disease.

21 January 2018

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Predictors of mortality in alpha1-antitrypsin deficiency

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Pneumococcal vaccination for patients with COPD: current practice and future directions.

21 January 2018

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Physiological and radiological characterisation of patients diagnosed with chronic obstructive pulmonary disease in primary care

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Prevalence and impact of bronchiectasis in alpha1-antitrypsin deficiency

21 January 2018

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Accumulation of PiZ alpha 1-antitrypsin causes liver damage in transgenic mice

21 January 2018

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Characterization of α1-Antitrypsin in the Inclusion Bodies from the Liver in α1-Antitrypsin Deficiency

21 January 2018

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Alpha1-antitrypsin deficiency in 26-year-old subjects: lung, liver, and protease/protease inhibitor studies

21 January 2018

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The liver in 30-year-old individuals with alpha(1)-antitrypsin deficiency

21 January 2018

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Late manifestation of chronic liver disease in adults with alpha-1-antitrypsin deficiency

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

21 January 2018

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Genetic modifiers of liver disease in cystic fibrosis

21 January 2018

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Alpha1-Antitrypsin Deficiency-Associated Panniculitis: Case Report and Review of the Literature

21 January 2018

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Chronic pancreatitis and alpha-1-antitrypsin

21 January 2018

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Strategies for dissecting genetic-environmental interactions in neurodegenerative disorders

21 January 2018

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Art, alpha-1-antitrypsin polymorphisms and intense creative energy: blessing or curse?

21 January 2018

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Genome scan of a second wave of NIMH genetics initiative bipolar pedigrees: chromosomes 2, 11, 13, 14, and X.

21 January 2018

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The serpin MNEI inhibits elastase-like and chymotrypsin-like serine proteases through efficient reactions at two active sites

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Structure and function of matrix metalloproteinases and TIMPs

21 January 2018

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A novel proteolytic cascade generates an extracellular matrix-derived chemoattractant in chronic neutrophilic inflammation

21 January 2018

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Matrix metalloproteinase-8 facilitates neutrophil migration through the corneal stromal matrix by collagen degradation and production of the chemotactic peptide Pro-Gly-Pro

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Complement receptor expression on neutrophils at an inflammatory site, the Pseudomonas-infected lung in cystic fibrosis

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Cleavage of CXCR1 on neutrophils disables bacterial killing in cystic fibrosis lung disease

21 January 2018

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

21 January 2018

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Neutrophil elastase up-regulates interleukin-8 via toll-like receptor 4.

21 January 2018

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

21 January 2018

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PAR-2 activation and LPS synergistically enhance inflammatory signaling in airway epithelial cells by raising PAR expression level and interleukin-8 release

21 January 2018

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Neutrophil elastase induces IL-8 gene transcription and protein release through p38/NF-{kappa}B activation via EGFR transactivation in a lung epithelial cell line

21 January 2018

1 reference

Dual oxidase 1-dependent MUC5AC mucin expression in cultured human airway epithelial cells

21 January 2018

1 reference

Activation of the epidermal growth factor receptor (EGFR) by a novel metalloprotease pathway.

21 January 2018

1 reference

Effect of pro-inflammatory stimuli on mucin expression and inhibition by secretory leucoprotease inhibitor.

21 January 2018

1 reference

Replacement therapy of alpha 1-antitrypsin deficiency. Reversal of protease-antiprotease imbalance within the alveolar structures of PiZ subjects

21 January 2018

1 reference

Replacement therapy for alpha 1-antitrypsin deficiency associated with emphysema

21 January 2018

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Does alpha1-antitrypsin augmentation therapy slow the annual decline in FEV1 in patients with severe hereditary alpha1-antitrypsin deficiency? Wissenschaftliche Arbeitsgemeinschaft zur Therapie von Lungenerkrankungen (WATL) alpha1-AT study group

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21 January 2018

1 reference

Exploring the role of CT densitometry: a randomised study of augmentation therapy in alpha1-antitrypsin deficiency.

21 January 2018

1 reference

Exploring the optimum approach to the use of CT densitometry in a randomised placebo-controlled study of augmentation therapy in alpha 1-antitrypsin deficiency

21 January 2018

1 reference

Therapeutic efficacy of alpha-1 antitrypsin augmentation therapy on the loss of lung tissue: an integrated analysis of 2 randomised clinical trials using computed tomography densitometry

21 January 2018

1 reference

Recombinant DNA-produced alpha 1-antitrypsin administered by aerosol augments lower respiratory tract antineutrophil elastase defenses in individuals with alpha 1-antitrypsin deficiency

21 January 2018

1 reference

Adenoviral gene delivery of elafin and secretory leukocyte protease inhibitor attenuates NF-kappa B-dependent inflammatory responses of human endothelial cells and macrophages to atherogenic stimuli

21 January 2018

1 reference

Anti-inflammatory and antimicrobial roles of secretory leukocyte protease inhibitor

21 January 2018

1 reference

Secretory leukocyte protease inhibitor suppresses the production of monocyte prostaglandin H synthase-2, prostaglandin E2, and matrix metalloproteinases

21 January 2018

1 reference

Suppression of macrophage responses to bacterial lipopolysaccharide (LPS) by secretory leukocyte protease inhibitor (SLPI) is independent of its anti-protease function

21 January 2018

1 reference

Modulation of airway inflammation in cystic fibrosis. In vivo suppression of interleukin-8 levels on the respiratory epithelial surface by aerosolization of recombinant secretory leukoprotease inhibitor

21 January 2018

1 reference

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

21 January 2018

1 reference

Z α-1 antitrypsin deficiency and the endoplasmic reticulum stress response

21 January 2018

1 reference

Protein Misfolding and Obstructive Lung Disease

21 January 2018

1 reference

Tauroursodeoxycholic acid inhibits apoptosis induced by Z alpha-1 antitrypsin via inhibition of Bad.

21 January 2018

1 reference

Anti-apoptotic effects of Z alpha1-antitrypsin in human bronchial epithelial cells.

21 January 2018

1 reference

Evidence for unfolded protein response activation in monocytes from individuals with alpha-1 antitrypsin deficiency

21 January 2018

1 reference

Accumulation of mutant alpha1-antitrypsin Z in the endoplasmic reticulum activates caspases-4 and -12, NFkappaB, and BAP31 but not the unfolded protein response.

21 January 2018

1 reference

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

21 January 2018

1 reference

Z alpha1-antitrypsin polymerizes in the lung and acts as a neutrophil chemoattractant

21 January 2018

1 reference

Polymers of Z alpha1-antitrypsin co-localize with neutrophils in emphysematous alveoli and are chemotactic in vivo

21 January 2018

1 reference

ER protein quality control and proteasome-mediated protein degradation

21 January 2018

1 reference

Molecular pathogenesis of alpha-1-antitrypsin deficiency-associated liver disease: a meeting review

21 January 2018

1 reference

Alpha-1-antitrypsin deficiency: importance of proteasomal and autophagic degradative pathways in disposal of liver disease-associated protein aggregates

21 January 2018

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21 January 2018

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21 January 2018

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21 January 2018

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The Atg8 and Atg12 ubiquitin-like conjugation systems in macroautophagy. 'Protein modifications: beyond the usual suspects' review series

21 January 2018

1 reference

The role of autophagy in alpha-1-antitrypsin deficiency: a specific cellular response in genetic diseases associated with aggregation-prone proteins

21 January 2018

1 reference

Mitochondrial autophagy and injury in the liver in alpha 1-antitrypsin deficiency.

21 January 2018

1 reference

Chronic obstructive pulmonary disease: towards pharmacogenetics

21 January 2018

1 reference

Phenotypic differences in alpha 1 antitrypsin-deficient sibling pairs may relate to genetic variation

21 January 2018

1 reference

Pi MZ and COPD: will we ever know?

21 January 2018

1 reference

Chronic obstructive pulmonary disease in alpha1-antitrypsin PI MZ heterozygotes: a meta-analysis

21 January 2018

1 reference

α₁-Antitrypsin protease inhibitor MZ heterozygosity is associated with airflow obstruction in two large cohorts

21 January 2018

1 reference

Neutrophilic inflammation and IL-8 levels in induced sputum of alpha-1-antitrypsin PiMZ subjects

21 January 2018

1 reference

CT scan appearance, densitometry, and health status in protease inhibitor SZ alpha1-antitrypsin deficiency.

21 January 2018

1 reference

The protease inhibitor PI*S allele and COPD: a meta-analysis

21 January 2018

1 reference

alpha1-Antitrypsin augmentation therapy for PI*MZ heterozygotes: a cautionary note.

21 January 2018

1 reference

alpha1-Antitrypsin deficiency and lung disease: risk modification by occupational and environmental inhalants.

21 January 2018

1 reference

American Thoracic Society/European Respiratory Society statement: standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency.

21 January 2018

1 reference

Antibody response to aerosolized transgenic human alpha1-antitrypsin.

21 January 2018

1 reference

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

21 January 2018

1 reference

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

21 January 2018

1 reference

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

21 January 2018

1 reference

Recombinant AAV serotype and capsid mutant comparison for pulmonary gene transfer of alpha-1-antitrypsin using invasive and noninvasive delivery

21 January 2018

1 reference

Preclinical characterization of a recombinant adeno-associated virus type 1-pseudotyped vector demonstrates dose-dependent injection site inflammation and dissemination of vector genomes to distant sites

21 January 2018

1 reference

Phase I trial of intramuscular injection of a recombinant adeno-associated virus serotype 2 alphal-antitrypsin (AAT) vector in AAT-deficient adults

21 January 2018

1 reference

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

21 January 2018

1 reference

Medicine. Clearing conformational disease

21 January 2018

1 reference

An autophagy-enhancing drug promotes degradation of mutant alpha1-antitrypsin Z and reduces hepatic fibrosis.

21 January 2018

1 reference

Selenoprotein S/SEPS1 modifies endoplasmic reticulum stress in Z variant alpha1-antitrypsin deficiency

21 January 2018

1 reference

Generation of transgene-free lung disease-specific human induced pluripotent stem cells using a single excisable lentiviral stem cell cassette.

21 January 2018

1 reference

A genome-wide association study in chronic obstructive pulmonary disease (COPD): identification of two major susceptibility loci.

21 January 2018

1 reference

S and Z alpha1-antitrypsin alleles are risk factors for bronchial hyperresponsiveness in young farmers: an example of gene/environment interaction

21 January 2018

1 reference

21 January 2018

Identifiers

DOI

10.1586/ERS.11.20

1 reference

24 August 2017

1 reference