Bezafibrate lowers very long-chain fatty acids in X-linked adrenoleukodystrophy fibroblasts by inhibiting fatty acid elongation

Journal of Inherited Metabolic Disease

Volumn 35, Issue 6, 2012, Pages 1137-1145

  Engelen, Marc ^a   Schackmann, Martin J A ^a   Ofman, Rob ^a   Sanders, Robert Jan ^a   Dijkstra, Inge M E ^a   Houten, Sander M ^a   Fourcade, Stéphane ^b   Pujol, Aurora ^b,c   Poll The, Bwee Tien ^a   Wanders, Ronald J A ^a   Kemp, Stephan ^a,d  

^a UNIVERSITY OF AMSTERDAM   (Netherlands)

^b CENTRO DE INVESTIGACIÓN BIOMÉDICA EN RED DE ENFERMEDADES RARAS CIBERER   (Spain)

^c INSTITUCIÓ CATALANA DE RECERCA I ESTUDIS AVANÇATS ICREA   (Spain)

^d ACADEMIC MEDICAL CENTER   (Netherlands)

Author keywords

[No Author keywords available]

Indexed keywords

BEZAFIBRATE; PEROXISOME PROLIFERATOR ACTIVATED RECEPTOR; VERY LONG CHAIN FATTY ACID;

ADRENOLEUKODYSTROPHY; ARTICLE; CHILDHOOD ADRENOLEUKODYSTROPHY; CONTROLLED STUDY; FATTY ACID CHAIN ELONGATION; FATTY ACID METABOLISM; FATTY ACID OXIDATION; FATTY ACID SYNTHESIS; FIBROBLAST; HUMAN; MOUSE; NONHUMAN; X CHROMOSOME LINKED DISORDER;

ACETYLTRANSFERASES; ADRENOLEUKODYSTROPHY; ANIMALS; ATP-BINDING CASSETTE TRANSPORTERS; BEZAFIBRATE; CELLS, CULTURED; ENZYME INHIBITORS; FATTY ACIDS; HUMANS; HYPOLIPIDEMIC AGENTS; MALE; MICE; MICE, TRANSGENIC; MICROSOMES, LIVER; OXIDATION-REDUCTION; PEROXISOME PROLIFERATOR-ACTIVATED RECEPTORS; RECOMBINANT PROTEINS;

EID: 84867872472     PISSN: 01418955     EISSN: 15732665     Source Type: Journal    
DOI: 10.1007/s10545-012-9471-4     Document Type: Article

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