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.
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Misfolded proteins partition between two distinct quality control compartmentsOne step at a time: endoplasmic reticulum-associated degradationEndoplasmic reticulum-associated degradation (ERAD) and autophagy cooperate to degrade polymerogenic mutant serpinsRecent technical developments in the study of ER-associated degradationMechanisms for quality control of misfolded transmembrane proteinsCapitalizing on the autophagic response for treatment of liver disease caused by alpha-1-antitrypsin deficiency and other genetic diseasesThe delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiologyFluphenazine reduces proteotoxicity in C. elegans and mammalian models of alpha-1-antitrypsin deficiencyDeficient and Null Variants of SERPINA1 Are Proteotoxic in a Caenorhabditis elegans Model of α1-Antitrypsin DeficiencyFutile protein folding cycles in the ER are terminated by the unfolded protein O-mannosylation pathway.ADD66, a gene involved in the endoplasmic reticulum-associated degradation of alpha-1-antitrypsin-Z in yeast, facilitates proteasome activity and assembly.A Role for Macro-ER-Phagy in ER Quality Control.FBG1 Is the Final Arbitrator of A1AT-Z DegradationAutophagy is activated for cell survival after endoplasmic reticulum stressNew Concepts in Alpha-1 Antitrypsin Deficiency Disease Mechanisms.ER-associated complexes (ERACs) containing aggregated cystic fibrosis transmembrane conductance regulator (CFTR) are degraded by autophagy.Autophagy counterbalances endoplasmic reticulum expansion during the unfolded protein responseRole of the ubiquitin proteasome system in Parkinson's disease.Free oligosaccharides to monitor glycoprotein endoplasmic reticulum-associated degradation in Saccharomyces cerevisiaeThe BiP molecular chaperone plays multiple roles during the biogenesis of torsinA, an AAA+ ATPase associated with the neurological disease early-onset torsion dystonia.Modularity of the Hrd1 ERAD complex underlies its diverse client range.Evasion of endoplasmic reticulum surveillance makes Wsc1p an obligate substrate of Golgi quality controlInterplay of substrate retention and export signals in endoplasmic reticulum quality control.Adiponectin modulates oxidative stress-induced mitophagy and protects C2C12 myoblasts against apoptosisQuality and quantity control at the endoplasmic reticulum.A C. elegans model of human α1-antitrypsin deficiency links components of the RNAi pathway to misfolded protein turnoverThe VCP/p97 system at a glance: connecting cellular function to disease pathogenesis.Activation of ERAD pathway by human hepatitis B virus modulates viral and subviral particle production.Molecular chaperones and substrate ubiquitination control the efficiency of endoplasmic reticulum-associated degradation.Role of autophagy in liver physiology and pathophysiology.Mitophagy: therapeutic potentials for liver disease and beyond.Protein misfolding and the serpinopathies.Mutant fibrinogen cleared from the endoplasmic reticulum via endoplasmic reticulum-associated protein degradation and autophagy: an explanation for liver disease.Protein folding and quality control in the endoplasmic reticulum: Recent lessons from yeast and mammalian cell systems.PiZ mouse liver accumulates polyubiquitin conjugates that associate with catalytically active 26S proteasomesEndoplasmic reticulum stress regulation of the Kar2p/BiP chaperone alleviates proteotoxicity via dual degradation pathways.Linking of autophagy to ubiquitin-proteasome system is important for the regulation of endoplasmic reticulum stress and cell viability.Screening for novel genes of Saccharomyces cerevisiae involved in recombinant antibody production.The endoplasmic reticulum-associated degradation pathways of budding yeast.The endosomal protein-sorting receptor sortilin has a role in trafficking α-1 antitrypsin
P2860
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P2860
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.
description
2005 nî lūn-bûn
@nan
2005 թուականի Նոյեմբերին հրատարակուած գիտական յօդուած
@hyw
2005 թվականի նոյեմբերին հրատարակված գիտական հոդված
@hy
2005年の論文
@ja
2005年論文
@yue
2005年論文
@zh-hant
2005年論文
@zh-hk
2005年論文
@zh-mo
2005年論文
@zh-tw
2005年论文
@wuu
name
Characterization of an ERAD ge ...... nother for aggregates of A1PiZ
@nl
Characterization of an ERAD ge ...... other for aggregates of A1PiZ.
@ast
Characterization of an ERAD ge ...... other for aggregates of A1PiZ.
@en
type
label
Characterization of an ERAD ge ...... nother for aggregates of A1PiZ
@nl
Characterization of an ERAD ge ...... other for aggregates of A1PiZ.
@ast
Characterization of an ERAD ge ...... other for aggregates of A1PiZ.
@en
prefLabel
Characterization of an ERAD ge ...... nother for aggregates of A1PiZ
@nl
Characterization of an ERAD ge ...... other for aggregates of A1PiZ.
@ast
Characterization of an ERAD ge ...... other for aggregates of A1PiZ.
@en
P2860
P356
P1476
Characterization of an ERAD ge ...... other for aggregates of A1PiZ.
@en
P2093
Jeffrey L Brodsky
P2860
P304
P356
10.1091/MBC.E04-09-0779
P407
P577
2005-11-02T00:00:00Z