Copper supplementation restores cytochrome c oxidase assembly defect in a mitochondrial disease model of COA6 deficiency

Human Molecular Genetics

Volumn 23, Issue 13, 2014, Pages 3596-3606

  Ghosh, Alok ^a   Trivedi, Prachi P ^a   Timbalia, Shrishiv A ^a   Griffin, Aaron T ^a   Rahn, Jennifer J ^b   Chan, Sherine S L ^b   Gohil, Vishal M ^a  

^a TEXAS A AND M UNIVERSITY   (United States)

^b MEDICAL UNIVERSITY OF SOUTH CAROLINA   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

COPPER; COPPER ZINC SUPEROXIDE DISMUTASE; CYTOCHROME C OXIDASE; MITOCHONDRIAL PROTEIN; SACCHAROMYCES CEREVISIAE PROTEIN;

ARTICLE; CONGENITAL HEART MALFORMATION; COPPER METABOLISM; CYTOCHROME C OXIDASE DEFICIENCY; DIET SUPPLEMENTATION; EMBRYO; FIBROBLAST; GENE FUNCTION; GENE MUTATION; HEART RATE; HUMAN; HUMAN CELL; HYPERTROPHIC CARDIOMYOPATHY; MITOCHONDRIAL RESPIRATION; MITOCHONDRION; NATIVE POLYACRYLAMIDE GEL ELECTROPHORESIS; NONHUMAN; PATHOGENICITY; PRIORITY JOURNAL; PROTEIN LOCALIZATION; PROTEIN MOTIF; SACCHAROMYCES CEREVISIAE; STEADY STATE; WESTERN BLOTTING; ZEBRA FISH; ANIMAL; CYTOLOGY; DISORDERS OF MITOCHONDRIAL FUNCTIONS; METABOLISM; MUTATION; SKIN;

ANIMALS; COPPER; ELECTRON TRANSPORT COMPLEX IV; HUMANS; MITOCHONDRIA; MITOCHONDRIAL DISEASES; MITOCHONDRIAL PROTEINS; MUTATION; SACCHAROMYCES CEREVISIAE; SACCHAROMYCES CEREVISIAE PROTEINS; SKIN; ZEBRAFISH;

EID: 84902313031     PISSN: 09646906     EISSN: 14602083     Source Type: Journal    
DOI: 10.1093/hmg/ddu069     Document Type: Article

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