A bifunctional protein with deficient enzymic activity: identification of a new peroxisomal disorder using novel methods to measure the peroxisomal beta-oxidation enzyme activities.
Wanders
R J
RJ
Department of Paediatrics, University Hospital Amsterdam, The Netherlands.
van Roermund
C W
CW
Schelen
A
A
Schutgens
R B
RB
Tager
J M
JM
Stephenson
J B
JB
Clayton
P T
PT
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
D009097:Multienzyme Complexes; D015094:3-Hydroxyacyl CoA Dehydrogenases; C576253:EHHADH protein, human; D004746:Enoyl-CoA Hydratase; D063994:Peroxisomal Bifunctional Enzyme; D007535:Isomerases