Correction of fatty acid oxidation in carnitine palmitoyl transferase 2-deficient cultured skin fibroblasts by bezafibrate

Carnitine palmitoyltransferase 2 (CPTII) deficiency is among the most common inborn errors of mitochondrial fatty acid beta-oxidation (FAO). Clinical phenotype varies in relation to the metabolic block, as assessed by studies of FAO in patient fibroblasts. Thus, fibroblasts from patients with mild m...

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Veröffentlicht in:	Pediatric research 2003-10, Vol.54 (4), p.446-451
Hauptverfasser:	DJOUADI, Fatima, BONNEFONT, Jean-Paul, THUILLIER, Laure, DRCIN, Véronique, KHADOM, Noman, MUNNICH, Arnold, BASTIN, Jean
Format:	Artikel
Sprache:	eng
Schlagworte:	Bezafibrate - pharmacology Biological and medical sciences Carnitine O-Palmitoyltransferase - deficiency Carnitine O-Palmitoyltransferase - genetics Carnitine O-Palmitoyltransferase - metabolism Cells, Cultured Dose-Response Relationship, Drug Fatty Acids - metabolism Fibroblasts - cytology Fibroblasts - drug effects Fibroblasts - metabolism Fundamental and applied biological sciences. Psychology General aspects Genetics of eukaryotes. Biological and molecular evolution Humans Hypolipidemic Agents - pharmacology Life Sciences Medical sciences Molecular and cellular biology Oxidation-Reduction Skin - cytology
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