title: Characterisation of a novel enzyme of human fatty acid beta-oxidation: a matrix-associated, mitochondrial 2-enoyl-CoA hydratase.
abstract: We present evidence for the existence of a previously unrecognised enzyme of mitochondrial beta-oxidation in man. This enzyme, which is situated in the mitochondrial matrix and has medium- and long-chain 2-enoyl-CoA hydratase activity, was identified by studying tissues from a patient with a severe deficiency of the trifunctional protein of mitochondrial beta-oxidation. The novel enzyme is present in all tissues studied--heart, liver and cultured skin fibroblasts--but is particularly active in liver. Together with other recently identified enzymes of mitochondrial beta-oxidation, the existence of this new enzyme suggests that two mitochondrial beta-oxidation enzyme systems exist in man: one associated with the mitochondrial membrane, responsible for the beta-oxidation of long-chain fatty acids and one in the mitochondrial matrix, responsible for the oxidation of medium- and short-chain fatty acids.
authors.affiliation: Division of Clinical Neuroscience, University of Newcastle upon Tyne, U.K.
authors.forename: S
authors.initials: S
authors.lastname: Jackson
authors.forename: J
authors.initials: J
authors.lastname: Schaefer
authors.forename: B
authors.initials: B
authors.lastname: Middleton
authors.forename: D M
authors.initials: DM
authors.lastname: Turnbull
mesh_terms: D002478:Cells, Cultured; D004746:Enoyl-CoA Hydratase / Q000737:chemistry / Q000378:metabolism*; D005227:Fatty Acids / Q000378:metabolism*; D006801:Humans; D008929:Mitochondria, Heart / Q000201:enzymology*; D008930:Mitochondria, Liver / Q000201:enzymology*; D008931:Mitochondria, Muscle / Q000201:enzymology*; D010084:Oxidation-Reduction; D013379:Substrate Specificity
chemical_list: D005227:Fatty Acids; D004746:Enoyl-CoA Hydratase