Formation of high molecular weight complexes of mutant Cu, Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis.
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ASK1 is essential for endoplasmic reticulum stress-induced neuronal cell death triggered by expanded polyglutamine repeatsParkin accumulation in aggresomes due to proteasome impairmentHeterozygous missense mutations in BSCL2 are associated with distal hereditary motor neuropathy and Silver syndromeBAG3 mediates chaperone-based aggresome-targeting and selective autophagy of misfolded proteinsHDAC6 regulates mutant SOD1 aggregation through two SMIR motifs and tubulin acetylationDisulfide cross-linked protein represents a significant fraction of ALS-associated Cu, Zn-superoxide dismutase aggregates in spinal cords of model micePrion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cellsStructures of the G85R variant of SOD1 in familial amyotrophic lateral sclerosisMisfolded CuZnSOD and amyotrophic lateral sclerosisDorfin localizes to the ubiquitylated inclusions in Parkinson's disease, dementia with Lewy bodies, multiple system atrophy, and amyotrophic lateral sclerosisALS-associated mutant SOD1G93A causes mitochondrial vacuolation by expansion of the intermembrane space and by involvement of SOD1 aggregation and peroxisomesIonic self-complementarity induces amyloid-like fibril formation in an isolated domain of a plant copper metallochaperone proteinSOD1 misplacing and mitochondrial dysfunction in amyotrophic lateral sclerosis pathogenesisMolecular motor proteins and amyotrophic lateral sclerosisMechanisms of mutant SOD1 induced mitochondrial toxicity in amyotrophic lateral sclerosisRecruitment of the oncoprotein v-ErbA to aggresomes.Tempol moderately extends survival in a hSOD1(G93A) ALS rat model by inhibiting neuronal cell loss, oxidative damage and levels of non-native hSOD1(G93A) formsAn alternative mechanism of bicarbonate-mediated peroxidation by copper-zinc superoxide dismutase: rates enhanced via proposed enzyme-associated peroxycarbonate intermediateThe complex molecular biology of amyotrophic lateral sclerosis (ALS)Protein disulphide isomerase protects against protein aggregation and is S-nitrosylated in amyotrophic lateral sclerosisInclusion body myopathy-associated mutations in p97/VCP impair endoplasmic reticulum-associated degradationThe rate and equilibrium constants for a multistep reaction sequence for the aggregation of superoxide dismutase in amyotrophic lateral sclerosisA faulty interaction between SOD1 and hCCS in neurodegenerative diseaseMetal-free superoxide dismutase-1 and three different amyotrophic lateral sclerosis variants share a similar partially unfolded beta-barrel at physiological temperatureMutant SOD1 instability: implications for toxicity in amyotrophic lateral sclerosis.Local unfolding in a destabilized, pathogenic variant of superoxide dismutase 1 observed with H/D exchange and mass spectrometry.Familial amyotrophic lateral sclerosis mutants of copper/zinc superoxide dismutase are susceptible to disulfide reduction.From structure to redox: The diverse functional roles of disulfides and implications in disease.Distinct partitioning of ALS associated TDP-43, FUS and SOD1 mutants into cellular inclusionsCharacterization of detergent-insoluble proteins in ALS indicates a causal link between nitrative stress and aggregation in pathogenesis.Analysis of the cytosolic proteome in a cell culture model of familial amyotrophic lateral sclerosis reveals alterations to the proteasome, antioxidant defenses, and nitric oxide synthetic pathways.Mitochondrial proteomic analysis of a cell line model of familial amyotrophic lateral sclerosis.No widespread induction of cell death genes occurs in pure motoneurons in an amyotrophic lateral sclerosis mouse model.Proteasomes remain intact, but show early focal alteration in their composition in a mouse model of amyotrophic lateral sclerosis.Protein aggregation and protein instability govern familial amyotrophic lateral sclerosis patient survival.Aggresomes do not represent a general cellular response to protein misfolding in mammalian cellsModulation of mutant superoxide dismutase 1 aggregation by co-expression of wild-type enzyme.Uncoupling the Trade-Off between Somatic Proteostasis and Reproduction in Caenorhabditis elegans Models of Polyglutamine Diseases.Protein Quality Control and the Amyotrophic Lateral Sclerosis/Frontotemporal Dementia ContinuumDNA-triggered aggregation of copper, zinc superoxide dismutase in the presence of ascorbate.
P2860
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P2860
Formation of high molecular weight complexes of mutant Cu, Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis.
description
2000 nî lūn-bûn
@nan
2000年の論文
@ja
2000年学术文章
@wuu
2000年学术文章
@zh
2000年学术文章
@zh-cn
2000年学术文章
@zh-hans
2000年学术文章
@zh-my
2000年学术文章
@zh-sg
2000年學術文章
@yue
2000年學術文章
@zh-hant
name
Formation of high molecular we ...... amyotrophic lateral sclerosis.
@ast
Formation of high molecular we ...... amyotrophic lateral sclerosis.
@en
type
label
Formation of high molecular we ...... amyotrophic lateral sclerosis.
@ast
Formation of high molecular we ...... amyotrophic lateral sclerosis.
@en
prefLabel
Formation of high molecular we ...... amyotrophic lateral sclerosis.
@ast
Formation of high molecular we ...... amyotrophic lateral sclerosis.
@en
P2093
P2860
P356
P1476
Formation of high molecular we ...... amyotrophic lateral sclerosis
@en
P2093
M E Gurney
M J Dalton
R R Kopito
P2860
P304
12571-12576
P356
10.1073/PNAS.220417997
P407
P577
2000-11-01T00:00:00Z