Overexpression of CCS in G93A-SOD1 mice leads to accelerated neurological deficits with severe mitochondrial pathology.
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Regulation of the copper chaperone CCS by XIAP-mediated ubiquitinationDecreased glutathione accelerates neurological deficit and mitochondrial pathology in familial ALS-linked hSOD1(G93A) mice modelStructures of the G85R variant of SOD1 in familial amyotrophic lateral sclerosisCopper Homeostasis as a Therapeutic Target in Amyotrophic Lateral Sclerosis with SOD1 MutationsSOD1 misplacing and mitochondrial dysfunction in amyotrophic lateral sclerosis pathogenesisMechanisms of mutant SOD1 induced mitochondrial toxicity in amyotrophic lateral sclerosisStructural and Biophysical Properties of the Pathogenic SOD1 Variant H46R/H48Q †Structural and biophysical properties of metal-free pathogenic SOD1 mutants A4V and G93ACopper delivery to the CNS by CuATSM effectively treats motor neuron disease in SOD(G93A) mice co-expressing the Copper-Chaperone-for-SODCopper metallochaperonesA faulty interaction between SOD1 and hCCS in neurodegenerative diseaseA comprehensive assessment of the SOD1G93A low-copy transgenic mouse, which models human amyotrophic lateral sclerosis.A random set scoring model for prioritization of disease candidate genes using protein complexes and data-mining of GeneRIF, OMIM and PubMed records.Modulation of mutant superoxide dismutase 1 aggregation by co-expression of wild-type enzyme.Redox susceptibility of SOD1 mutants is associated with the differential response to CCS over-expression in vivo.A role for copper in the toxicity of zinc-deficient superoxide dismutase to motor neurons in amyotrophic lateral sclerosisImmature copper-zinc superoxide dismutase and familial amyotrophic lateral sclerosis.Biological effects of CCS in the absence of SOD1 enzyme activation: implications for disease in a mouse model for ALS.Effects of natural antioxidants in neurodegenerative disease.Mitochondrial dysfunction and intracellular calcium dysregulation in ALSGlutaredoxin 2 prevents aggregation of mutant SOD1 in mitochondria and abolishes its toxicity.Aggregation modulating elements in mutant human superoxide dismutase 1.Expression of a pathogenic mutation of SOD1 sensitizes aprataxin-deficient cells and mice to oxidative stress and triggers hallmarks of premature ageing.Aggregation propensities of superoxide dismutase G93 hotspot mutants mirror ALS clinical phenotypesCupric ions induce the oxidation and trigger the aggregation of human superoxide dismutase 1Motor neuron trophic factors: therapeutic use in ALS?Effect of CCS on the accumulation of FALS SOD1 mutant-containing aggregates and on mitochondrial translocation of SOD1 mutants: implication of a free radical hypothesisBiochemical properties and in vivo effects of the SOD1 zinc-binding site mutant (H80G)The role of environmental exposures in neurodegeneration and neurodegenerative diseases.Rosmarinic Acid Alleviates Neurological Symptoms in the G93A-SOD1 Transgenic Mouse Model of Amyotrophic Lateral Sclerosis.Isolated cytochrome c oxidase deficiency in G93A SOD1 mice overexpressing CCS proteinDifferent regulation of wild-type and mutant Cu,Zn superoxide dismutase localization in mammalian mitochondria.Role of mutant SOD1 disulfide oxidation and aggregation in the pathogenesis of familial ALSTherapeutics for Alzheimer's disease based on the metal hypothesisMitochondria in neuroplasticity and neurological disorders.The right to choose: multiple pathways for activating copper,zinc superoxide dismutase.Mutant SOD1 in neuronal mitochondria causes toxicity and mitochondrial dynamics abnormalities.Mitochondrial function, morphology, and axonal transport in amyotrophic lateral sclerosis.Abnormal intracellular calcium signaling and SNARE-dependent exocytosis contributes to SOD1G93A astrocyte-mediated toxicity in amyotrophic lateral sclerosis.CuII(atsm) improves the neurological phenotype and survival of SOD1G93A mice and selectively increases enzymatically active SOD1 in the spinal cord
P2860
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P2860
Overexpression of CCS in G93A-SOD1 mice leads to accelerated neurological deficits with severe mitochondrial pathology.
description
2007 nî lūn-bûn
@nan
2007年の論文
@ja
2007年論文
@yue
2007年論文
@zh-hant
2007年論文
@zh-hk
2007年論文
@zh-mo
2007年論文
@zh-tw
2007年论文
@wuu
2007年论文
@zh
2007年论文
@zh-cn
name
Overexpression of CCS in G93A- ...... evere mitochondrial pathology.
@ast
Overexpression of CCS in G93A- ...... evere mitochondrial pathology.
@en
type
label
Overexpression of CCS in G93A- ...... evere mitochondrial pathology.
@ast
Overexpression of CCS in G93A- ...... evere mitochondrial pathology.
@en
prefLabel
Overexpression of CCS in G93A- ...... evere mitochondrial pathology.
@ast
Overexpression of CCS in G93A- ...... evere mitochondrial pathology.
@en
P2093
P2860
P356
P1476
Overexpression of CCS in G93A- ...... evere mitochondrial pathology.
@en
P2093
Bhagya Rajendran
Giovanni Manfredi
Hibiki Kawamata
Jeffrey L Elliott
Krishna Puttaparthi
Marjatta Son
Philip J Boyer
P2860
P304
P356
10.1073/PNAS.0610923104
P407
P577
2007-03-26T00:00:00Z