Bezafibrate increases very-long-chain acyl-CoA dehydrogenase protein and mRNA expression in deficient fibroblasts and is a potential therapy for fatty acid oxidation disorders

Human Molecular Genetics

Volumn 14, Issue 18, 2005, Pages 2695-2703

  Djouadi, F ^a   Aubey, F ^a   Schlemmer, D ^a   Ruiter, J P N ^b   Wanders, R J A ^c   Strauss, A W ^c   Bastin, Jean ^a  

^a HÔPITAL NECKER ENFANTS MALADES   (France)

^b ACADEMIC MEDICAL CENTER   (Netherlands)

^c MONROE CARELL JR CHILDREN S HOSPITAL AT VANDERBILT   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

BEZAFIBRATE; CARNITINE PALMITOYLTRANSFERASE; FENOFIBRIC ACID; FIBRIC ACID DERIVATIVE; LONG CHAIN ACYL COENZYME A DEHYDROGENASE; MESSENGER RNA; MUTANT PROTEIN; PALMITIC ACID; PEROXISOME PROLIFERATOR ACTIVATED RECEPTOR AGONIST;

ADOPTIVE IMMUNOTHERAPY; ARTICLE; CELL CULTURE; CONCENTRATION RESPONSE; CULTURE MEDIUM; DISEASE ASSOCIATION; DISEASE SEVERITY; ENZYME ACTIVITY; ENZYME DEFICIENCY; ENZYME INHIBITION; FATTY ACID OXIDATION; GENE EXPRESSION REGULATION; GENE MUTATION; HUMAN; HUMAN CELL; NEWBORN SCREENING; PRIORITY JOURNAL; PROTEIN ANALYSIS; PROTEIN FUNCTION; PROTEIN SYNTHESIS INHIBITION; REVERSE TRANSCRIPTION POLYMERASE CHAIN REACTION; SKIN FIBROBLAST; WESTERN BLOTTING;

ACYL-COA DEHYDROGENASE, LONG-CHAIN; BEZAFIBRATE; BLOTTING, WESTERN; CARNITINE; DNA PRIMERS; DOSE-RESPONSE RELATIONSHIP, DRUG; FIBROBLASTS; GENE EXPRESSION REGULATION, ENZYMOLOGIC; HUMANS; LIPID METABOLISM, INBORN ERRORS; MITOCHONDRIA; MUTATION, MISSENSE; PALMITATES; PEROXISOME PROLIFERATOR-ACTIVATED RECEPTORS; REVERSE TRANSCRIPTASE POLYMERASE CHAIN REACTION; RNA, MESSENGER;

EID: 24944563136     PISSN: 09646906     EISSN: None     Source Type: Journal    
DOI: 10.1093/hmg/ddi303     Document Type: Article

References (25)

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