Alpha1-antitrypsin polymerization and the serpinopathies: pathobiology and prospects for therapy - Wikidata - thesis-toulmin - TriplyDB

Alpha1-antitrypsin polymerization and the serpinopathies: pathobiology and prospects for therapy

Alpha1-antitrypsin polymerization and the serpinopathies: pathobiology and prospects for therapy

http://www.wikidata.org/entity/Q24554310

about

Serine protease inhibitors serpina1 and serpina3 are down-regulated in bone marrow during hematopoietic progenitor mobilization 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.Liver injury in alpha1-antitrypsin deficiency: an aggregated protein induces mitochondrial injury α-1 Antitrypsin regulates human neutrophil chemotaxis induced by soluble immune complexes and IL-8 Small molecules block the polymerization of Z alpha1-antitrypsin and increase the clearance of intracellular aggregates A surfactant protein C precursor protein BRICHOS domain mutation causes endoplasmic reticulum stress, proteasome dysfunction, and caspase 3 activation Megsin gene: its genomic analysis, pathobiological functions, and therapeutic perspectives Endoplasmic reticulum-associated degradation (ERAD) and autophagy cooperate to degrade polymerogenic mutant serpins The N terminus of the serpin, tengpin, functions to trap the metastable native state Models of chronic obstructive pulmonary disease Prolastin, a pharmaceutical preparation of purified human alpha1-antitrypsin, blocks endotoxin-mediated cytokine release Environmental, occupational, and genetic risk factors for alpha-1 antitrypsin deficiency.Neuroserpin Portland (Ser52Arg) is trapped as an inactive intermediate that rapidly forms polymers: implications for the epilepsy seen in the dementia FENIB Expression of hereditary hemochromatosis C282Y HFE protein in HEK293 cells activates specific endoplasmic reticulum stress responses.Exogenous alpha 1-antitrypsin down-regulates SERPINA1 expression Alpha-1 antitrypsin Pi*SZ genotype: estimated prevalence and number of SZ subjects worldwide.Survival prediction for pancreatic cancer patients receiving gemcitabine treatment.Altered native stability is the dominant basis for susceptibility of α1-antitrypsin mutants to polymerization.N-glycosylation and biological activity of recombinant human alpha1-antitrypsin expressed in a novel human neuronal cell line.The significance of the F variant of alpha-1-antitrypsin and unique case report of a PiFF homozygote Severe alpha-1 antitrypsin deficiency in composite heterozygotes inheriting a new splicing mutation QOMadrid Identification of a rare p.G320R alpha-1-antitrypsin variant in emphysema and lung cancer patients Protein aggregation in disease: a role for folding intermediates forming specific multimeric interactions.Protein misfolding and the serpinopathies.Z α-1 antitrypsin deficiency and the endoplasmic reticulum stress response.Orchestrating the unfolded protein response in health and disease.Mutant fibrinogen cleared from the endoplasmic reticulum via endoplasmic reticulum-associated protein degradation and autophagy: an explanation for liver disease.α-1-antitrypsin variants and the proteinase/antiproteinase imbalance in chronic obstructive pulmonary disease.Practical genetics: alpha-1-antitrypsin deficiency and the serpinopathies.Proteomic analysis of the serum in patients with idiopathic pulmonary arterial hypertension.Polymers of Z alpha1-antitrypsin co-localize with neutrophils in emphysematous alveoli and are chemotactic in vivo Alpha1-antitrypsin deficiency. 4: Molecular pathophysiology Alternative transcripts of the SERPINA1 gene in alpha-1 antitrypsin deficiency Global proteome profiling of dental cementum under experimentally-induced apposition.Matrix metalloproteinases in destructive pulmonary pathology.Pathogenesis of chronic liver injury and hepatocellular carcinoma in alpha-1-antitrypsin deficiency.Pulmonary emphysema: when more is less.The intracellular accumulation of polymeric neuroserpin explains the severity of the dementia FENIB.The hepatitis E virus ORF3 protein modulates epidermal growth factor receptor trafficking, STAT3 translocation, and the acute-phase response.Potential for therapeutic manipulation of the UPR in disease.

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Alpha1-antitrypsin polymerization and the serpinopathies: pathobiology and prospects for therapy

http://www.wikidata.org/entity/Q24554310

description

2002 nî lūn-bûn

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2002 թուականի Դեկտեմբերին հրատարակուած գիտական յօդուած

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2002 թվականի դեկտեմբերին հրատարակված գիտական հոդված

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2002年の論文

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2002年論文

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Alpha1-antitrypsin polymerizat ...... logy and prospects for therapy

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Alpha1-antitrypsin polymerizat ...... logy and prospects for therapy

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Alpha1-antitrypsin polymerizat ...... logy and prospects for therapy

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Alpha1-antitrypsin polymerizat ...... logy and prospects for therapy

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Alpha1-antitrypsin polymerizat ...... logy and prospects for therapy

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Journal of Clinical Investigation

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Alpha1-antitrypsin polymerizat ...... logy and prospects for therapy

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David A Lomas

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Ravi Mahadeva

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P2860

Liver injury in alpha1-antitrypsin deficiency: an aggregated protein induces mitochondrial injury

Inactive conformation of the serpin alpha(1)-antichymotrypsin indicates two-stage insertion of the reactive loop: implications for inhibitory function and conformational disease

Topography of a 2.0 Å structure of α1-antitrypsin reveals targets for rational drug design to prevent conformational disease

Inhibitory conformation of the reactive loop of alpha 1-antitrypsin

Familial dementia caused by polymerization of mutant neuroserpin

Mutant Neuroserpin (S49P) that causes familial encephalopathy with neuroserpin inclusion bodies is a poor proteinase inhibitor and readily forms polymers in vitro

What do dysfunctional serpins tell us about molecular mobility and disease?

Evolving concepts in the pathogenesis of chronic obstructive pulmonary disease.

The Z type variation of human alpha 1-antitrypsin causes a protein folding defect.

Dissecting glycoprotein quality control in the secretory pathway.

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1585-90

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scholarly article

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4581929

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10.1172/JCI0216782

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10.1172/JCI16782

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11

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110

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12464660

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151637

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