A novel HPLC-based method to diagnose peroxisomal D-bifunctional protein enoyl-CoA hydratase deficiency

Journal of Lipid Research

Volumn 44, Issue 3, 2003, Pages 640-644

  Gloerich, Jolein ^a   Denis, Simone ^a   Van Grunsven, Elisabeth G ^a   Dacremont, Georges ^b   Wanders, Ronald J A ^a   Ferdinandusse, Sacha ^a  

^a UNIVERSITY OF AMSTERDAM   (Netherlands)

^b GHENT UNIVERSITY   (Belgium)

Author keywords

Peroxisomal fatty acid oxidation disorders; Trihydroxycholestanoic acid; Very long chain fatty acids

Indexed keywords

BILE ACID; CHOLIC ACID; ENOYL COENZYME A HYDRATASE; 3 HYDROXYACYL COENZYME A DEHYDROGENASE; HSD17B4 PROTEIN, HUMAN; HYDROLYASE; HYDROXYSTEROID DEHYDROGENASE; MULTIENZYME COMPLEX;

ANALYTIC METHOD; ARTICLE; CLINICAL ARTICLE; CONTROLLED STUDY; D BIFUNCTIONAL PROTEIN ENOYL COENZYME A HYDRATASE DEFICIENCY; DISORDERS OF PEROXISOMAL FUNCTIONS; ENZYME ACTIVITY; ENZYME DEFICIENCY; ENZYME SUBSTRATE; FATTY ACID OXIDATION; FIBROBLAST; HIGH PERFORMANCE LIQUID CHROMATOGRAPHY; HUMAN; HUMAN CELL; MEASUREMENT; PRIORITY JOURNAL; PROTEIN DOMAIN; RELIABILITY; SACCHAROMYCES CEREVISIAE; YEAST; CELL LINE; ENZYMOLOGY; IMMUNOBLOTTING; IMMUNOLOGY; METHODOLOGY; PEROXISOME;

17-HYDROXYSTEROID DEHYDROGENASES; 3-HYDROXYACYL COA DEHYDROGENASES; CELL LINE; CHROMATOGRAPHY, HIGH PRESSURE LIQUID; ENOYL-COA HYDRATASE; FIBROBLASTS; HUMANS; HYDRO-LYASES; IMMUNOBLOTTING; MULTIENZYME COMPLEXES; PEROXISOMAL DISORDERS; PEROXISOMES; YEASTS;

EID: 0038281513     PISSN: 00222275     EISSN: None     Source Type: Journal    
DOI: 10.1194/jlr.D200039-JLR200     Document Type: Article

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