Mitochondrial dysfunction in mut methylmalonic acidemia

FASEB Journal

Volumn 23, Issue 4, 2009, Pages 1252-1261

  Chandler, Randy J ^a   Zerfas, Patricia M ^b   Shanske, Sara ^c   Sloan, Jennifer ^a   Hoffmann, Victoria ^b   DiMauro, Salvatore ^c   Venditti, Charles P ^a  

^a NATIONAL HUMAN GENOME RESEARCH INSTITUTE   (United States)

^b NATIONAL INSTITUTES OF HEALTH   (United States)

^c NewYork Presbyterian Hospital   (United States)

Author keywords

Cytochrome c oxidase; Glutathione; Methylmalonyl CoA mutase; Oxidant stress; Vitamin B12

Indexed keywords

CYTOCHROME C OXIDASE; GLUTATHIONE; LIVER EXTRACT; METHYLMALONYL COENZYME A MUTASE;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CASE REPORT; CHILD; CONTROLLED STUDY; DISORDERS OF MITOCHONDRIAL FUNCTIONS; ENZYME ACTIVITY; HUMAN; HUMAN TISSUE; INTERSTITIAL NEPHRITIS; KIDNEY DISEASE; KIDNEY PROXIMAL TUBULE; LIVER CELL; MALE; METHYLMALONIC ACIDEMIA; MORPHOLOGY; MOUSE; MUTANT; NONHUMAN; OXIDATIVE STRESS; PRIORITY JOURNAL; RESPIRATORY CHAIN; TIME; TISSUE SPECIFICITY; ANIMAL; C57BL MOUSE; CROSS BREEDING; DISEASE MODEL; DISORDERS OF AMINO ACID AND PROTEIN METABOLISM; GENETICS; HOMOZYGOTE; METABOLISM; MITOCHONDRION; MOUSE MUTANT; MUTATION; PRESCHOOL CHILD; ULTRASTRUCTURE;

ANIMALIA; MURINAE; MUS;

AMINO ACID METABOLISM, INBORN ERRORS; ANIMALS; CHILD, PRESCHOOL; CROSSES, GENETIC; DISEASE MODELS, ANIMAL; ELECTRON TRANSPORT COMPLEX IV; GLUTATHIONE; HOMOZYGOTE; HUMANS; MALE; METHYLMALONYL-COA MUTASE; MICE; MICE, INBRED C57BL; MICE, KNOCKOUT; MITOCHONDRIA; MUTATION; OXIDATIVE STRESS;

EID: 65349127550     PISSN: 08926638     EISSN: 15306860     Source Type: Journal    
DOI: 10.1096/fj.08-121848     Document Type: Article

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