The proteasome participates in degradation of mutant alpha 1-antitrypsin Z in the endoplasmic reticulum of hepatoma-derived hepatocytes.
about
MMP13 mutation causes spondyloepimetaphyseal dysplasia, Missouri type (SEMD(MO)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 therapyLiver injury in alpha1-antitrypsin deficiency: an aggregated protein induces mitochondrial injurySmall molecules block the polymerization of Z alpha1-antitrypsin and increase the clearance of intracellular aggregatesEndoplasmic reticulum-associated degradation (ERAD) and autophagy cooperate to degrade polymerogenic mutant serpinsCapitalizing 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 physiologyDeficient and Null Variants of SERPINA1 Are Proteotoxic in a Caenorhabditis elegans Model of α1-Antitrypsin DeficiencyMutants of neuroserpin that cause dementia accumulate as polymers within the endoplasmic reticulumFBG1 Is the Final Arbitrator of A1AT-Z DegradationNew Concepts in Alpha-1 Antitrypsin Deficiency Disease Mechanisms.Identification of ERGIC-53 as an intracellular transport receptor of alpha1-antitrypsin.Ubiquitin ligase SYVN1/HRD1 facilitates degradation of the SERPINA1 Z variant/α-1-antitrypsin Z variant via SQSTM1/p62-dependent selective autophagy.Modeling inherited metabolic disorders of the liver using human induced pluripotent stem cells.Hepatic fibrinogen storage disease due to the fibrinogen γ375 Arg → Trp mutation "fibrinogen Aguadilla" is present in ArabsMutant fibrinogen cleared from the endoplasmic reticulum via endoplasmic reticulum-associated protein degradation and autophagy: an explanation for liver disease.Therapeutic targeting of misfolding and conformational change in α1-antitrypsin deficiency.PiZ mouse liver accumulates polyubiquitin conjugates that associate with catalytically active 26S proteasomesAlpha1-antitrypsin deficiency. 4: Molecular pathophysiologyActivating transcription factor 6 limits intracellular accumulation of mutant α(1)-antitrypsin Z and mitochondrial damage in hepatoma cells.Linking of autophagy to ubiquitin-proteasome system is important for the regulation of endoplasmic reticulum stress and cell viability.Versatility of the endoplasmic reticulum protein folding factory.Pathogenesis of chronic liver injury and hepatocellular carcinoma in alpha-1-antitrypsin deficiency.Update on research, diagnosis and management of alphal-antitrypsin deficiency.Rapamycin reduces intrahepatic alpha-1-antitrypsin mutant Z protein polymers and liver injury in a mouse modelPolymers and inflammation: disease mechanisms of the serpinopathiesAutophagic disposal of the aggregation-prone protein that causes liver inflammation and carcinogenesis in alpha-1-antitrypsin deficiency.Single nucleotide polymorphism-mediated translational suppression of endoplasmic reticulum mannosidase I modifies the onset of end-stage liver disease in alpha1-antitrypsin deficiency.Endoplasmic reticulum polymers impair luminal protein mobility and sensitize to cellular stress in alpha1-antitrypsin deficiencyAn antibody that prevents serpin polymerisation acts by inducing a novel allosteric behaviour.α1-antitrypsin Deficiency: A Misfolded Secretory Protein Variant with Unique Effects on the Endoplasmic Reticulum.Hepatic 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.Liver disease in alpha-1 antitrypsin deficiency: current understanding and future therapy.Broad spectrum of hepatocyte inclusions in humans, animals, and experimental models.Advances in alpha-1-antitrypsin deficiency liver disease.Alpha-1 antitrypsin and liver disease: mechanisms of injury and novel interventions.Mechanism and Regulation of Autophagy and Its Role in Neuronal Diseases.Aberrant disulphide bonding contributes to the ER retention of alpha1-antitrypsin deficiency variants.
P2860
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P2860
The proteasome participates in degradation of mutant alpha 1-antitrypsin Z in the endoplasmic reticulum of hepatoma-derived hepatocytes.
description
2001 nî lūn-bûn
@nan
2001年の論文
@ja
2001年学术文章
@wuu
2001年学术文章
@zh-cn
2001年学术文章
@zh-hans
2001年学术文章
@zh-my
2001年学术文章
@zh-sg
2001年學術文章
@yue
2001年學術文章
@zh
2001年學術文章
@zh-hant
name
The proteasome participates in ...... hepatoma-derived hepatocytes.
@en
type
label
The proteasome participates in ...... hepatoma-derived hepatocytes.
@en
prefLabel
The proteasome participates in ...... hepatoma-derived hepatocytes.
@en
P2093
P356
P1476
The proteasome participates in ...... hepatoma-derived hepatocytes.
@en
P2093
D H Perlmutter
J H Teckman
P304
44865-44872
P356
10.1074/JBC.M103703200
P407
P577
2001-09-27T00:00:00Z