Copper supplementation restores cytochrome c oxidase activity in cultured cells from patients with SCO2 mutations - sprookjes - yvandenbroek - TriplyDB

Copper supplementation restores cytochrome c oxidase activity in cultured cells from patients with SCO2 mutations

Copper supplementation restores cytochrome c oxidase activity in cultured cells from patients with SCO2 mutations

http://www.wikidata.org/entity/Q24534079

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Unexpected vascular enrichment of SCO1 over SCO2 in mammalian tissues: implications for human mitochondrial disease Analysis of mouse models of cytochrome c oxidase deficiency owing to mutations in Sco2 Molecular mechanisms of HIF-1alpha modulation induced by oxygen tension and BMP2 in glioblastoma derived cells.Modulation of mitochondrial protein phosphorylation by soluble adenylyl cyclase ameliorates cytochrome oxidase defects.A functionally dominant mitochondrial DNA mutation.Essential role for Atox1 in the copper-mediated intracellular trafficking of the Menkes ATPase.How periplasmic thioredoxin TlpA reduces bacterial copper chaperone ScoI and cytochrome oxidase subunit II (CoxB) prior to metallation.Mitochondrial complex IV deficiency, caused by mutated COX6B1, is associated with encephalomyopathy, hydrocephalus and cardiomyopathy.A targetable fluorescent sensor reveals that copper-deficient SCO1 and SCO2 patient cells prioritize mitochondrial copper homeostasis.Mitochondrial encephalomyopathies.Tetramethylpyrazine (TMP) protects against sodium arsenite-induced nephrotoxicity by suppressing ROS production, mitochondrial dysfunction, pro-inflammatory signaling pathways and programed cell death.The roles of Rhodobacter sphaeroides copper chaperones PCu(A)C and Sco (PrrC) in the assembly of the copper centers of the aa(3)-type and the cbb(3)-type cytochrome c oxidases Copper and bezafibrate cooperate to rescue cytochrome c oxidase deficiency in cells of patients with SCO2 mutations.PGC-1-related coactivator complexes with HCF-1 and NRF-2beta in mediating NRF-2(GABP)-dependent respiratory gene expression.Suppression mechanisms of COX assembly defects in yeast and human: insights into the COX assembly process Evidence for nuclear modifier gene in mitochondrial cardiomyopathy.Identification of Small Molecule Inhibitors of Human Cytochrome c Oxidase That Target Chemoresistant Glioma Cells.The m.3243A>G mtDNA mutation is pathogenic in an in vitro model of the human blood brain barrier.Mutated PET117 causes complex IV deficiency and is associated with neurodevelopmental regression and medulla oblongata lesions.The mitochondrion: a central architect of copper homeostasis.Mutation in mitochondrial complex IV subunit COX5A causes pulmonary arterial hypertension, lactic acidemia, and failure to thrive.

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P2860

Copper supplementation restores cytochrome c oxidase activity in cultured cells from patients with SCO2 mutations

http://www.wikidata.org/entity/Q24534079

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2002 nî lūn-bûn

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2002 թուականի Ապրիլին հրատարակուած գիտական յօդուած

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2002 թվականի ապրիլին հրատարակված գիտական հոդված

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2002年の論文

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Copper supplementation restore ...... m patients with SCO2 mutations

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Copper supplementation restore ...... m patients with SCO2 mutations

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Copper supplementation restore ...... m patients with SCO2 mutations

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Copper supplementation restore ...... m patients with SCO2 mutations

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Copper supplementation restore ...... m patients with SCO2 mutations

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Copper supplementation restore ...... m patients with SCO2 mutations

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Copper supplementation restore ...... m patients with SCO2 mutations

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Copper supplementation restore ...... m patients with SCO2 mutations

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Copper supplementation restore ...... m patients with SCO2 mutations

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Eric A Schon

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Evelyn Hernandez-Rosa

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Leonardo Salviati

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Mercy M Davidson

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Sabrina Sacconi

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Winsome F Walker

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P2860

SURF1, encoding a factor involved in the biogenesis of cytochrome c oxidase, is mutated in Leigh syndrome

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

A mutation in the human heme A:farnesyltransferase gene (COX10 ) causes cytochrome c oxidase deficiency

The copper chaperone for superoxide dismutase

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

Shy1p occurs in a high molecular weight complex and is required for efficient assembly of cytochrome c oxidase in yeast.

Cox11p is required for stable formation of the Cu(B) and magnesium centers of cytochrome c oxidase.

Characterization of COX17, a yeast gene involved in copper metabolism and assembly of cytochrome oxidase.

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

Cytochrome c oxidase deficiency due to mutations in SCO2, encoding a mitochondrial copper-binding protein, is rescued by copper in human myoblasts

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10.1042/BJ3630321

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P433

Pt 2

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363

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Salvatore DiMauro

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2002-04-15T00:00:00Z

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11931660

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P932

1222481

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