Inhibition of 3-methylcrotonyl-CoA carboxylase explains the increased excretion of 3-hydroxyisovaleric acid in valproate-treated patients

Journal of Inherited Metabolic Disease

Volumn 35, Issue 3, 2012, Pages 443-449

  Luís, Paula B M ^a   Ruiter, Jos P ^b   IJlst, Lodewijk ^b   Diogo, Luísa ^c   Garcia, Paula ^c   De Almeida, Isabel Tavares ^a   Duran, Marinus ^b   Wanders, Ronald J ^b   Silva, Margarida F B ^a  

^a RESEARCH INSTITUTE FOR MEDICINES IMED ULISBOA   (Portugal)

^b ACADEMIC MEDICAL CENTER   (Netherlands)

^c CENTRO HOSPITALAR E UNIVERSITÁRIO DE COIMBRA   (Portugal)

Author keywords

[No Author keywords available]

Indexed keywords

3 HYDROXYISOVALERIC ACID; 3 HYDROXYISOVALERYL COENZYME A; ALIPHATIC CARBOXYLIC ACID; BIOTINIDASE; COENZYME A; DRUG METABOLITE; ENOYL COENZYME A HYDRATASE; LEUCINE; METHYLCROTONOYL COENZYME A CARBOXYLASE; UNCLASSIFIED DRUG; VALPROIC ACID; VALPROYL COENZYME A;

ARTICLE; CONTROLLED STUDY; DRUG EFFECT; ENZYME ACTIVITY; ENZYME INHIBITION; HUMAN; HUMAN TISSUE; IC 50; LIVER HOMOGENATE; URINE LEVEL;

BIOTINIDASE; CARBON-CARBON LIGASES; CASE-CONTROL STUDIES; CHROMATOGRAPHY, HIGH PRESSURE LIQUID; ENZYME INHIBITORS; GAS CHROMATOGRAPHY-MASS SPECTROMETRY; HUMANS; INHIBITORY CONCENTRATION 50; KINETICS; LEUCINE; LIVER; METABOLOMICS; MODELS, CHEMICAL; VALERATES; VALPROIC ACID;

EID: 84863308641     PISSN: 01418955     EISSN: 15732665     Source Type: Journal    
DOI: 10.1007/s10545-011-9423-4     Document Type: Article

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