AMINO-ACID AND NUCLEOTIDE-SEQUENCES OF HUMAN PEROXISOMAL ENOYL-COA HYDRATASE - 3-HYDROXYACYL-COA DEHYDROGENASE CDNA

Deficiency of enoyl-CoA hydratase : 3-hydroxyacyl-CoA dehydrogenase (p eroxisomal bifunctional enzyme), one of the enzymes of the peroxisomal beta-oxidation system, leads to clinical manifestations resembling Ze llweger syndrome with hypotonia, psychomotor delay, hepatomegaly, typi cal facial appearance and accumulation of very long-chain fatty acids. The nucleotide sequence of the human peroxisomal enoyl-CoA hydratase : 3-hydroxyacyl-CoA dehydrogenase cDNA has been reported by Hoefler an d colleagues; however, we have found some amino acid differences from our originally isolated cDNA. Contrary to the findings described in a previous paper, we report here the cDNA sequence of human peroxisomal enoyl-CoA hydratase : 3-hydroxyacyl-CoA dehydrogenase in which there a re 9 authenticated amino acid alterations.