title: A bifunctional protein with deficient enzymic activity: identification of a new peroxisomal disorder using novel methods to measure the peroxisomal beta-oxidation enzyme activities.
authors.affiliation: Department of Paediatrics, University Hospital Amsterdam, The Netherlands.
authors.forename: R J
authors.initials: RJ
authors.lastname: Wanders
authors.forename: C W
authors.initials: CW
authors.lastname: van Roermund
authors.forename: A
authors.initials: A
authors.lastname: Schelen
authors.forename: R B
authors.initials: RB
authors.lastname: Schutgens
authors.forename: J M
authors.initials: JM
authors.lastname: Tager
authors.forename: J B
authors.initials: JB
authors.lastname: Stephenson
authors.forename: P T
authors.initials: PT
authors.lastname: Clayton
mesh_terms: D015094:3-Hydroxyacyl CoA Dehydrogenases / Q000172:deficiency*; D020642:Acatalasia; D002458:Cell Fractionation; D002478:Cells, Cultured; D004746:Enoyl-CoA Hydratase / Q000172:deficiency*; D005347:Fibroblasts / Q000648:ultrastructure*; D006801:Humans; D007535:Isomerases; D008830:Microbodies / Q000201:enzymology*; D009097:Multienzyme Complexes / Q000172:deficiency*; D010084:Oxidation-Reduction; D063994:Peroxisomal Bifunctional Enzyme; D015211:Zellweger Syndrome / Q000201:enzymology
chemical_list: D009097:Multienzyme Complexes; D015094:3-Hydroxyacyl CoA Dehydrogenases; C576253:EHHADH protein, human; D004746:Enoyl-CoA Hydratase; D063994:Peroxisomal Bifunctional Enzyme; D007535:Isomerases