Bifunctional protein deficiency: complementation within the same group suggesting differential enzyme defects and clues to the underlying basis

J Inherit Metab Dis. 1998 Jun;21(3):298-301. doi: 10.1023/a:1005396912015.

Authors

E G Van Grunsven¹, E van Berkel, H Lemonde, P T Clayton, R J Wanders

Affiliation

¹ University of Amsterdam, Academic Medical Center, Department of Clinical Chemistry, The Netherlands.

PMID: 9686380
DOI: 10.1023/a:1005396912015

No abstract available

MeSH terms

3-Hydroxyacyl CoA Dehydrogenases / metabolism*
Cells, Cultured
Enoyl-CoA Hydratase / metabolism*
Humans
Isomerases*
Multienzyme Complexes / metabolism*
Peroxisomal Bifunctional Enzyme
Peroxisomal Disorders / enzymology*

Substances

Multienzyme Complexes
3-Hydroxyacyl CoA Dehydrogenases
EHHADH protein, human
Enoyl-CoA Hydratase
Peroxisomal Bifunctional Enzyme
Isomerases