Structural analysis of cDNAs for subunits of human mitochondrial fatty acid beta-oxidation trifunctional protein.

Abstract

Trifunctional protein deficiency, a typical mitochondrial long-chain fatty acid beta-oxidation defect, is caused by the abnormality of mitochondrial long-chain enoyl-CoA hydratase/3-hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase trifunctional protein consisting of four moles of alpha-subunit and four moles of beta-subunit. We cloned, sequenced, and expressed the following cDNAs for the alpha- and beta-subunits of human trifunctional protein. The 2,690-bp cDNA clone had a 2,289-bp open reading frame encoding a 82,958-Da precursor and a 78,969-Da mature subunit (alpha-subunit). Expression of this cDNA in mammalian cells yielded a polypeptide with the long-chain enoyl-CoA hydratase and long-chain 3-hydroxyacyl-CoA dehydrogenase activities. The 1,991-bp cDNA clone had a 1,422-bp open reading frame encoding a 51,293-Da precursor and a 47,484-Da mature subunit (beta-subunit). Expression of this cDNA in mammalian cells yielded a polypeptide with the long-chain 3-ketoacyl-CoA thiolase activity.

• Authors: T Kamijo, T Aoyama, A Komiyama, T Hashimoto
• Journal: Biochemical and biophysical research communications (Biochem Biophys Res Commun) Vol. 199 Issue 2 Pg. 818-25 (Mar 15 1994) ISSN: 0006-291X [Print] United States
• PMID: 8135828 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Codon
• DNA, Complementary
• Macromolecular Substances
• Multienzyme Complexes
• RNA, Messenger
• Fatty Acid Desaturases
• Mitochondrial Trifunctional Protein

Topics

• Amino Acid Sequence
• Animals
• Base Sequence
• Blotting, Northern
• Cell Line
• Codon (genetics)
• DNA, Complementary (analysis)
• Fatty Acid Desaturases (metabolism)
• Genetic Vectors
• Humans
• Macromolecular Substances
• Mammals
• Mitochondria (metabolism)
• Mitochondria, Liver (metabolism)
• Mitochondrial Trifunctional Protein
• Molecular Sequence Data
• Multienzyme Complexes (biosynthesis, genetics, metabolism)
• RNA, Messenger (analysis, metabolism)
• Restriction Mapping
• Transfection