Role of ubiquitin in proteasomal degradation of mutant alpha(1)-antitrypsin Z in the endoplasmic reticulum.
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SERPINA2 is a novel gene with a divergent function from SERPINA1RNF185 is a novel E3 ligase of endoplasmic reticulum-associated degradation (ERAD) that targets cystic fibrosis transmembrane conductance regulator (CFTR)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 injuryThe delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiologyFBG1 Is the Final Arbitrator of A1AT-Z DegradationUbiquitin ligase SYVN1/HRD1 facilitates degradation of the SERPINA1 Z variant/α-1-antitrypsin Z variant via SQSTM1/p62-dependent selective autophagy.Inhibition of endoplasmic reticulum-associated degradation in CHO cells resistant to cholera toxin, Pseudomonas aeruginosa exotoxin A, and ricin.Role of autophagy in liver physiology and pathophysiology.Role of Drosophila EDEMs in the degradation of the alpha-1-antitrypsin Z variant.PiZ mouse liver accumulates polyubiquitin conjugates that associate with catalytically active 26S proteasomesActivating transcription factor 6 limits intracellular accumulation of mutant α(1)-antitrypsin Z and mitochondrial damage in hepatoma cells.Sequestration of mutated alpha1-antitrypsin into inclusion bodies is a cell-protective mechanism to maintain endoplasmic reticulum function.Autophagy: A protective mechanism in response to stress and inflammation.Rapamycin reduces intrahepatic alpha-1-antitrypsin mutant Z protein polymers and liver injury in a mouse modelHepatic fibrosis and carcinogenesis in α1-antitrypsin deficiency: a prototype for chronic tissue damage in gain-of-function disorders.Novel treatment strategies for liver disease due to α1-antitrypsin deficiency.p62 Is a common component of cytoplasmic inclusions in protein aggregation diseases.Mitochondrial autophagy and injury in the liver in alpha 1-antitrypsin deficiency.Autophagy induced by exogenous bile acids is therapeutic in a model of α-1-AT deficiency liver disease.
P2860
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P2860
Role of ubiquitin in proteasomal degradation of mutant alpha(1)-antitrypsin Z in the endoplasmic reticulum.
description
2000 nî lūn-bûn
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2000年の論文
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2000年学术文章
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2000年学术文章
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2000年学术文章
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2000年学术文章
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2000年学术文章
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2000年學術文章
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2000年學術文章
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name
Role of ubiquitin in proteasom ...... in the endoplasmic reticulum.
@en
type
label
Role of ubiquitin in proteasom ...... in the endoplasmic reticulum.
@en
prefLabel
Role of ubiquitin in proteasom ...... in the endoplasmic reticulum.
@en
P2093
P2860
P1476
Role of ubiquitin in proteasom ...... in the endoplasmic reticulum.
@en
P2093
Perlmutter DH
Teckman JH
P2860
P304
P356
10.1152/AJPGI.2000.278.1.G39
P577
2000-01-01T00:00:00Z