[HTML][HTML] Hepatocellular and hepatic peroxisomal alterations in mice with a disrupted peroxisomal fatty acyl-coenzyme A oxidase gene

CY Fan, J Pan, R Chu, D Lee, KD Kluckman… - Journal of Biological …, 1996 - ASBMB
Peroxisomal genetic disorders, such as Zellweger syndrome, are characterized by defects in
one or more enzymes involved in the peroxisomal β-oxidation of very long chain fatty acids
and are associated with defective peroxisomal biogenesis. The biologic role of peroxisomal
β-oxidation system, which consists of three enzymes: fatty acyl-CoA oxidase (ACOX), enoyl-
CoA hydratase/3-hydroxyacyl-CoA dehydrogenase (HD), and thiolase, has been examined
in mice by disrupting ACOX gene, which encodes the first and rate-limiting enzyme of this …