alpha1-Antitrypsin deficiency, chronic obstructive pulmonary disease and the serpinopathies. - Wikidata - wikimedia - TriplyDB

alpha1-Antitrypsin deficiency, chronic obstructive pulmonary disease and the serpinopathies.

alpha1-Antitrypsin deficiency, chronic obstructive pulmonary disease and the serpinopathies.

http://www.wikidata.org/entity/Q37474870

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The delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiology Genome wide association identifies common variants at the SERPINA6/SERPINA1 locus influencing plasma cortisol and corticosteroid binding globulin Malfolded protein structure and proteostasis in lung diseases.The endoplasmic reticulum protein folding factory and its chaperones: new targets for drug discovery?Protease Inhibitors in Tick Saliva: The Role of Serpins and Cystatins in Tick-host-Pathogen Interaction.Altered native stability is the dominant basis for susceptibility of α1-antitrypsin mutants to polymerization.The shapes of Z-α1-antitrypsin polymers in solution support the C-terminal domain-swap mechanism of polymerization.A novel SERPINA1 mutation causing serum alpha(1)-antitrypsin deficiency.Chaperoning osteogenesis: new protein-folding disease paradigms.Neutrophil elastase inhibitors.Graphene oxide as an enzyme inhibitor: modulation of activity of α-chymotrypsin.A blood fluke serine protease inhibitor regulates an endogenous larval elastase.NOX2 As a Target for Drug Development: Indications, Possible Complications, and Progress.Quantitative assessment of the impact of the gut microbiota on lysine epsilon-acetylation of host proteins using gnotobiotic mice Encapsulation of alpha-1 antitrypsin in PLGA nanoparticles: in vitro characterization as an effective aerosol formulation in pulmonary diseases.Molecular Mechanism of Z α1-Antitrypsin Deficiency Cause-specific mortality in individuals with severe alpha 1-antitrypsin deficiency in comparison with the general population in Sweden.Antisense oligonucleotide treatment ameliorates alpha-1 antitrypsin-related liver disease in mice.Twenty years of polymers: a personal perspective on alpha-1 antitrypsin deficiency.Recombinant production of native human α-1-antitrypsin protein in the liver HepG2 cells.Protein accumulation in the endoplasmic reticulum as a non-equilibrium phase transition.Osteoblast Malfunction Caused by Cell Stress Response to Procollagen Misfolding in α2(I)-G610C Mouse Model of Osteogenesis Imperfecta.Development of Bullous Disease during Treatment of Pulmonary Marginal Zone B-Cell Lymphoma.Using antisense technology to develop a novel therapy for α-1 antitrypsin deficient (AATD) liver disease and to model AATD lung disease.Proteostasis: a new therapeutic paradigm for pulmonary disease.Reactive centre loop mutants of α-1-antitrypsin reveal position-specific effects on intermediate formation along the polymerization pathway.Knockdown of Z Mutant Alpha-1 Antitrypsin In Vivo Using Modified DNA Antisense Oligonucleotides.Liver Disease in Alpha-1 Antitrypsin Deficiency: Current Approaches and Future Directions.

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alpha1-Antitrypsin deficiency, chronic obstructive pulmonary disease and the serpinopathies.

http://www.wikidata.org/entity/Q37474870

description

article científic

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article scientifique

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articolo scientifico

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artigo científico

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bilimsel makale

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scientific article published on 14 May 2009

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vedecký článok

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vetenskaplig artikel

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videnskabelig artikel

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vědecký článek

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name

alpha1-Antitrypsin deficiency, ...... isease and the serpinopathies.

@en

alpha1-Antitrypsin deficiency, ...... isease and the serpinopathies.

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type

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alpha1-Antitrypsin deficiency, ...... isease and the serpinopathies.

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alpha1-Antitrypsin deficiency, ...... isease and the serpinopathies.

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alpha1-Antitrypsin deficiency, ...... isease and the serpinopathies.

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alpha1-Antitrypsin deficiency, ...... isease and the serpinopathies.

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Clinical Science

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alpha1-Antitrypsin deficiency, ...... isease and the serpinopathies.

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Damian C Crowther

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David A Lomas

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Didier Belorgey

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Heike Kroger

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Ian MacLeod

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Juan Pérez

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Peter Hägglöf

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Susanna Karlsson-Li

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Ugo I Ekeowa

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P2860

Structure of a serpin-protease complex shows inhibition by deformation

Role of EDEM in the release of misfolded glycoproteins from the calnexin cycle

Human EDEM2, a novel homolog of family 47 glycosidases, is involved in ER-associated degradation of glycoproteins

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.

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

Small molecules block the polymerization of Z alpha1-antitrypsin and increase the clearance of intracellular aggregates

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

A 2.1 A resolution structure of an uncleaved alpha(1)-antitrypsin shows variability of the reactive center and other loops

Serpin polymerization is prevented by a hydrogen bond network that is centered on his-334 and stabilized by glycerol

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837-850

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scholarly article

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10.1042/CS20080484

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12

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116

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Stefan J Marciniak

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2009-05-14T00:00:00Z

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19426146

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chronic obstructive pulmonary disease