Endoplasmic reticulum polymers impair luminal protein mobility and sensitize to cellular stress in alpha1-antitrypsin deficiency
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Deficient and Null Variants of SERPINA1 Are Proteotoxic in a Caenorhabditis elegans Model of α1-Antitrypsin DeficiencyAFM Imaging Reveals Topographic Diversity of Wild Type and Z Variant Polymers of Human α1-Proteinase InhibitorNew Concepts in Alpha-1 Antitrypsin Deficiency Disease Mechanisms.Neuroserpin polymers cause oxidative stress in a neuronal model of the dementia FENIBERAD defects and the HFE-H63D variant are associated with increased risk of liver damages in Alpha 1-Antitrypsin DeficiencyThe role of bronchial epithelial cells in the pathogenesis of COPD in Z-alpha-1 antitrypsin deficiencyTherapeutic targeting of misfolding and conformational change in α1-antitrypsin deficiency.A dynamic study of protein secretion and aggregation in the secretory pathway.A single-chain variable fragment intrabody prevents intracellular polymerization of Z α1-antitrypsin while allowing its antiproteinase activityIntroduction: the unfolded protein response's role in disease pathophysiology.Biochemical nature of Russell Bodies.Interactions between N-linked glycosylation and polymerisation of neuroserpin within the endoplasmic reticulumNetrin-1 Protects Hepatocytes Against Cell Death Through Sustained Translation During the Unfolded Protein ResponseThe endoplasmic reticulum remains functionally connected by vesicular transport after its fragmentation in cells expressing Z-α1-antitrypsin.Probing endoplasmic reticulum dynamics using fluorescence imaging and photobleaching techniques.An antibody that prevents serpin polymerisation acts by inducing a novel allosteric behaviour.Broad spectrum of hepatocyte inclusions in humans, animals, and experimental models.The integrated stress response in lung disease.Characterising the association of latency with α(1)-antitrypsin polymerisation using a novel monoclonal antibodyIncreased ERK signalling promotes inflammatory signalling in primary airway epithelial cells expressing Z α1-antitrypsinAn antibody raised against a pathogenic serpin variant induces mutant-like behaviour in the wild-type protein.Emergence of a stage-dependent human liver disease signature with directed differentiation of alpha-1 antitrypsin-deficient iPS cells.Electrophoresis- and FRET-Based Measures of Serpin Polymerization.Proteomic analysis of plasma from rats following total parenteral nutrition-induced liver injury.α1-Antitrypsin deficiency.
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P2860
Endoplasmic reticulum polymers impair luminal protein mobility and sensitize to cellular stress in alpha1-antitrypsin deficiency
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 March 2013
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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
Endoplasmic reticulum polymers ...... alpha1-antitrypsin deficiency
@en
Endoplasmic reticulum polymers ...... alpha1-antitrypsin deficiency.
@nl
type
label
Endoplasmic reticulum polymers ...... alpha1-antitrypsin deficiency
@en
Endoplasmic reticulum polymers ...... alpha1-antitrypsin deficiency.
@nl
prefLabel
Endoplasmic reticulum polymers ...... alpha1-antitrypsin deficiency
@en
Endoplasmic reticulum polymers ...... alpha1-antitrypsin deficiency.
@nl
P2093
P2860
P50
P356
P1433
P1476
Endoplasmic reticulum polymers ...... alpha1-antitrypsin deficiency
@en
P2093
Adriana Ordóñez
David A Lomas
P2860
P304
P356
10.1002/HEP.26173
P407
P577
2013-03-14T00:00:00Z