Amino acid and nucleotide sequences of human peroxisomal enoyl-CoA hydratase: 3-hydroxyacyl-CoA dehydrogenase cDNA.

Abstract

Deficiency of enoyl-CoA hydratase: 3-hydroxyacyl-CoA dehydrogenase (peroxisomal bifunctional enzyme), one of the enzymes of the peroxisomal beta-oxidation system, leads to clinical manifestations resembling Zellweger syndrome with hypotonia, psychomotor delay, hepatomegaly, typical 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 and 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 are 9 authenticated amino acid alterations.

• Authors: S Fukuda, Y Suzuki, N Shimozawa, Z Zhang, T Orii, T Aoyama, T Hashimoto, N Kondo
• Journal: Journal of inherited metabolic disease (J Inherit Metab Dis) Vol. 21 Issue 1 Pg. 23-8 (Feb 1998) ISSN: 0141-8955 [Print] United States
• PMID: 9501266 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

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

Topics

• 3-Hydroxyacyl CoA Dehydrogenases (genetics)
• Base Sequence
• DNA, Complementary
• Enoyl-CoA Hydratase (genetics)
• Humans
• Isomerases
• Molecular Sequence Data
• Multienzyme Complexes (genetics)
• Peroxisomal Bifunctional Enzyme
• Sequence Analysis, DNA