Increased superoxide accumulation in pyruvate dehydrogenase complex deficient fibroblasts

Molecular Genetics and Metabolism

Volumn 104, Issue 3, 2011, Pages 255-260

  Glushakova, Lyudmyla G ^a   Judge, Sharon ^a   Cruz, Alex ^a   Pourang, Deena ^a   Mathews, Clayton E ^a   Stacpoole, Peter W ^a  

^a UNIVERSITY OF FLORIDA COLLEGE OF MEDICINE   (United States)

Author keywords

Human PDC deficiency; Mitochondria; Oxidative stress; Reactive oxygen nitrogen species

Indexed keywords

CELL MARKER; HYPOXIA INDUCIBLE FACTOR 1ALPHA; LACTIC ACID; MANGANESE SUPEROXIDE DISMUTASE; PYRUVATE DEHYDROGENASE COMPLEX; SUPEROXIDE; UNCOUPLING PROTEIN 2;

ADULT; ARTICLE; BIOACCUMULATION; CELL GROWTH; CELL VIABILITY; CLINICAL ARTICLE; CONTROLLED STUDY; DNA DAMAGE; ENZYME ACTIVITY; ENZYME PHOSPHORYLATION; EXON; FEMALE; GENE DISRUPTION; GENE MUTATION; HUMAN; HUMAN CELL; HUMAN TISSUE; LACTIC ACIDOSIS; MALE; MISSENSE MUTATION; MITOCHONDRIAL ENERGY TRANSFER; OXIDATIVE STRESS; PRIORITY JOURNAL; PROTEIN EXPRESSION; PYRUVATE DEHYDROGENASE COMPLEX DEFICIENCY; SKIN FIBROBLAST;

BLOTTING, WESTERN; CELLS, CULTURED; DNA DAMAGE; ELECTRON TRANSPORT CHAIN COMPLEX PROTEINS; FIBROBLASTS; HUMANS; HYPOXIA-INDUCIBLE FACTOR 1, ALPHA SUBUNIT; ION CHANNELS; MITOCHONDRIAL PROTEINS; OXIDATIVE STRESS; PYRUVATE DEHYDROGENASE (LIPOAMIDE); PYRUVATE DEHYDROGENASE COMPLEX DEFICIENCY DISEASE; REACTIVE OXYGEN SPECIES; SKIN; SUPEROXIDE DISMUTASE; SUPEROXIDES;

EID: 82455164221     PISSN: 10967192     EISSN: 10967206     Source Type: Journal    
DOI: 10.1016/j.ymgme.2011.07.023     Document Type: Article

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