Beta-oxidation of long-chain fatty acids by human fibroblasts: evidence for a novel long-chain acyl-coenzyme A dehydrogenase.

Abstract

Fibroblasts from patients with long-chain acyl-CoA dehydrogenase deficiency were found to oxidize [1-14C]linoleate at an average rate of 60% of normal but [9,10(n)-3H]myristate at an average rate of only 37% of normal, a relationship reverse from that predicted by the chain-length specificities of the three known straight-chain mitochondrial acyl-CoA dehydrogenases. The residual long-chain beta-oxidative activity was found to be mitochondrial and associated with the accumulation of tetradecadienoate (C14:2w6) when the mutant fibroblasts were incubated with 100 mumol/L linoleate (C18:2w6) or eicosadienoate (C20:2w6). The results suggest the presence in human fibroblasts of a novel acyl-CoA dehydrogenase with activity toward 15 to 20 carbon-length fatty acids.

• Authors: R I Kelley
• Journal: Biochemical and biophysical research communications (Biochem Biophys Res Commun) Vol. 182 Issue 3 Pg. 1002-7 (Feb 14 1992) ISSN: 0006-291X [Print] United States
• PMID: 1540149 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Carbon Radioisotopes
• Linoleic Acids
• Myristic Acids
• Myristic Acid
• Tritium
• Linoleic Acid
• Acyl-CoA Dehydrogenase
• Acyl-CoA Dehydrogenase, Long-Chain

Topics

• Acyl-CoA Dehydrogenase
• Acyl-CoA Dehydrogenase, Long-Chain (deficiency, metabolism)
• Carbon Radioisotopes
• Cell Line
• Cells, Cultured
• Fibroblasts (enzymology)
• Humans
• Kinetics
• Linoleic Acid
• Linoleic Acids (metabolism)
• Myristic Acid
• Myristic Acids (metabolism)
• Oxidation-Reduction
• Skin (enzymology)
• Substrate Specificity
• Tritium