Secondary coenzyme Q10 deficiency and oxidative stress in cultured fibroblasts from patients with riboflavin responsive multiple Acyl-CoA dehydrogenation deficiency

Human Molecular Genetics

Volumn 22, Issue 19, 2013, Pages 3819-3827

  Cornelius, Nanna ^a,b   Byron, Colleen ^c   Hargreaves, Iain ^d   Guerra, Paula Fernandez ^a,b   Furdek, Andrea K ^c   Land, John ^d   Radford, Weston W ^c   Frerman, Frank ^e   Corydon, Thomas J ^b   Gregersen, Niels ^a,b   Olsen, Rikke K J ^a,b  

^a AARHUS UNIVERSITY HOSPITAL   (Denmark)

^b AARHUS UNIVERSITY   (Denmark)

^c RIPON COLLEGE   (United States)

^d National Hospital   (United Kingdom)

^e Children's Hospital Colorado   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ACYL COENZYME A; AMINO ACID; ELECTRON TRANSFERRING FLAVOPROTEIN; FATTY ACID; FLAVINE ADENINE NUCLEOTIDE; OXYGEN; REACTIVE OXYGEN METABOLITE; RIBOFLAVIN; SUCCINATE DEHYDROGENASE (UBIQUINONE); UBIDECARENONE;

ARTICLE; CELL CULTURE; CONTROLLED STUDY; ELECTRON TRANSPORT; ENERGY YIELD; FATTY ACID OXIDATION; FIBROBLAST; HUMAN; HUMAN CELL; IN VITRO STUDY; MULTIPLE ACYL COA DEHYDROGENASE DEFICIENCY; OXIDATIVE STRESS; PRIORITY JOURNAL; PROTEIN BINDING; RESPIRATORY CHAIN; RHODOBACTER SPHAEROIDES; WILD TYPE;

ACYL COENZYME A; ATAXIA; BACTERIAL PROTEINS; CELLS, CULTURED; ELECTRON-TRANSFERRING FLAVOPROTEINS; FIBROBLASTS; GENETIC VARIATION; HUMANS; IRON-SULFUR PROTEINS; MITOCHONDRIA; MITOCHONDRIAL DISEASES; MULTIPLE ACYL COENZYME A DEHYDROGENASE DEFICIENCY; MUSCLE WEAKNESS; OXIDATION-REDUCTION; OXIDATIVE STRESS; OXIDOREDUCTASES ACTING ON CH-NH GROUP DONORS; REACTIVE OXYGEN SPECIES; RHODOBACTER SPHAEROIDES; RIBOFLAVIN; UBIQUINONE;

EID: 84888815418     PISSN: 09646906     EISSN: 14602083     Source Type: Journal    
DOI: 10.1093/hmg/ddt232     Document Type: Article

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