The pleiotropic effects of decanoic acid treatment on mitochondrial function in fibroblasts from patients with complex I deficient Leigh syndrome

Journal of Inherited Metabolic Disease

Volumn 39, Issue 3, 2016, Pages 415-426

  Kanabus, Marta ^a   Fassone, Elisa ^a   Hughes, Sean David ^a,b   Bilooei, Sara Farahi ^a   Rutherford, Tricia ^c   O’Donnell, Maura ^c   Heales, Simon J R ^a,b,d   Rahman, Shamima ^a,b  

^a UNIVERSITY COLLEGE LONDON   (United Kingdom)

^b GREAT ORMOND STREET HOSPITAL   (United Kingdom)

^c Vitaflo International Ltd   (United Kingdom)

^d NATIONAL HOSPITAL FOR NEUROLOGY AND NEUROSURGERY   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

CARNITINE PALMITOYLTRANSFERASE I; CATALASE; CITRATE SYNTHASE; CYTOCHROME C OXIDASE; DECANOIC ACID; FATTY ACID; LONG CHAIN ACYL COENZYME A DEHYDROGENASE; PEROXISOME PROLIFERATOR ACTIVATED RECEPTOR GAMMA; PHOSPHOENOLPYRUVATE CARBOXYKINASE (GTP); PHOSPHOENOLPYRUVATE CARBOXYKINASE 2; PYRUVATE DEHYDROGENASE KINASE; PYRUVATE DEHYDROGENASE KINASE 4; PYRUVATE DEHYDROGENASE KINASE ISOZYME 3; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE DEHYDROGENASE (UBIQUINONE); UNCLASSIFIED DRUG; VERY LONG CHAIN ACYL COENZYME A DEHYDROGENASE; DECANOIC ACID DERIVATIVE; GLUCOSE;

ARTICLE; CELL FUNCTION; COMPLEX I DEFICIENCY; CONTROLLED STUDY; DOWN REGULATION; ENZYME ACTIVITY; ENZYME ASSAY; FATTY ACID METABOLISM; FIBROBLAST; FLOW CYTOMETRY; GENE EXPRESSION; GLUCOSE METABOLISM; HUMAN; HUMAN CELL; LEIGH DISEASE; MICROARRAY ANALYSIS; MITOCHONDRIAL RESPIRATION; MITOCHONDRION; OXIDATIVE STRESS; POLYMERASE CHAIN REACTION; PROTEIN EXPRESSION; RESPIRATORY CHAIN; UPREGULATION; CELL CULTURE; DISORDERS OF MITOCHONDRIAL FUNCTIONS; DRUG EFFECTS; KETOGENIC DIET; METABOLISM; PROCEDURES;

CELLS, CULTURED; DECANOIC ACIDS; DIET, KETOGENIC; ELECTRON TRANSPORT COMPLEX I; FATTY ACIDS; FIBROBLASTS; GENE EXPRESSION; GLUCOSE; HUMANS; LEIGH DISEASE; MITOCHONDRIA; MITOCHONDRIAL DISEASES;

EID: 84963765159     PISSN: 01418955     EISSN: 15732665     Source Type: Journal    
DOI: 10.1007/s10545-016-9930-4     Document Type: Article

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