Isolated cytochrome c oxidase deficiency in G93A SOD1 mice overexpressing CCS protein - Wikidata - wikimedia - TriplyDB

Isolated cytochrome c oxidase deficiency in G93A SOD1 mice overexpressing CCS protein

Isolated cytochrome c oxidase deficiency in G93A SOD1 mice overexpressing CCS protein

http://www.wikidata.org/entity/Q36727229

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SOD1 misplacing and mitochondrial dysfunction in amyotrophic lateral sclerosis pathogenesis Copper delivery to the CNS by CuATSM effectively treats motor neuron disease in SOD(G93A) mice co-expressing the Copper-Chaperone-for-SOD "Pulling the plug" on cellular copper: the role of mitochondria in copper export.Redox susceptibility of SOD1 mutants is associated with the differential response to CCS over-expression in vivo.Effects of natural antioxidants in neurodegenerative disease.Mitochondrial dysfunction and intracellular calcium dysregulation in ALS Glutaredoxin 2 prevents aggregation of mutant SOD1 in mitochondria and abolishes its toxicity.Biochemical properties and in vivo effects of the SOD1 zinc-binding site mutant (H80G)In vivo pathogenic role of mutant SOD1 localized in the mitochondrial intermembrane space Different regulation of wild-type and mutant Cu,Zn superoxide dismutase localization in mammalian mitochondria.The Psi(m) depolarization that accompanies mitochondrial Ca2+ uptake is greater in mutant SOD1 than in wild-type mouse motor terminals.The right to choose: multiple pathways for activating copper,zinc superoxide dismutase.Abnormal intracellular calcium signaling and SNARE-dependent exocytosis contributes to SOD1G93A astrocyte-mediated toxicity in amyotrophic lateral sclerosis.Import, maturation, and function of SOD1 and its copper chaperone CCS in the mitochondrial intermembrane space.Mitochondrial dysfunction in familial amyotrophic lateral sclerosis.Neuronal calcium signaling: function and dysfunction.The ER mitochondria calcium cycle and ER stress response as therapeutic targets in amyotrophic lateral sclerosis.Mitochondria and endoplasmic reticulum crosstalk in amyotrophic lateral sclerosis.Targeting mitochondrial metal dyshomeostasis for the treatment of neurodegeneration.Insights into the mechanisms of copper dyshomeostasis in amyotrophic lateral sclerosis.Oxygen-dependent activation of Cu,Zn-superoxide dismutase-1.

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Isolated cytochrome c oxidase deficiency in G93A SOD1 mice overexpressing CCS protein

http://www.wikidata.org/entity/Q36727229

description

2008 nî lūn-bûn

@nan

2008年の論文

@ja

2008年論文

@yue

2008年論文

@zh-hant

2008年論文

@zh-hk

2008年論文

@zh-mo

2008年論文

@zh-tw

2008年论文

@wuu

2008年论文

@zh

2008年论文

@zh-cn

name

Isolated cytochrome c oxidase deficiency in G93A SOD1 mice overexpressing CCS protein

@ast

Isolated cytochrome c oxidase deficiency in G93A SOD1 mice overexpressing CCS protein

@en

type

label

Isolated cytochrome c oxidase deficiency in G93A SOD1 mice overexpressing CCS protein

@ast

Isolated cytochrome c oxidase deficiency in G93A SOD1 mice overexpressing CCS protein

@en

prefLabel

Isolated cytochrome c oxidase deficiency in G93A SOD1 mice overexpressing CCS protein

@ast

Isolated cytochrome c oxidase deficiency in G93A SOD1 mice overexpressing CCS protein

@en

P1433

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Journal of Biological Chemistry

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Isolated cytochrome c oxidase deficiency in G93A SOD1 mice overexpressing CCS protein

@en

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Dennis R Winge

http://www.w3.org/2001/XMLSchema#string

Jeffrey L Elliott

http://www.w3.org/2001/XMLSchema#string

Marjatta Son

http://www.w3.org/2001/XMLSchema#string

Nadine Romain

http://www.w3.org/2001/XMLSchema#string

Ronald G Haller

http://www.w3.org/2001/XMLSchema#string

Scot C Leary

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P2860

Fatal infantile cardioencephalomyopathy with COX deficiency and mutations in SCO2, a COX assembly gene

Cytochrome c oxidase and mitochondrial F1F0-ATPase (ATP synthase) activities in platelets and brain from patients with Alzheimer's disease

Mutations in COX10 result in a defect in mitochondrial heme A biosynthesis and account for multiple, early-onset clinical phenotypes associated with isolated COX deficiency

Mutations in COX15 produce a defect in the mitochondrial heme biosynthetic pathway, causing early-onset fatal hypertrophic cardiomyopathy

Mutations of the SCO1 gene in mitochondrial cytochrome c oxidase deficiency with neonatal-onset hepatic failure and encephalopathy.

Tissue-specific cytochrome c oxidase assembly defects due to mutations in SCO2 and SURF1

Rett syndrome: clinical review and genetic update

Involvement of mitochondrial ferredoxin and Cox15p in hydroxylation of heme O.

Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis

Mutations in SCO2 are associated with a distinct form of hypertrophic cardiomyopathy and cytochrome c oxidase deficiency

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12267-12275

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scholarly article

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10.1074/JBC.M708523200

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18

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2008-03-11T00:00:00Z

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18334481

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P932

2431012

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