Lignoceroyl-CoASH ligase: enzyme defect in fatty acid beta-oxidation system in X-linked childhood adrenoleukodystrophy.

Abstract

We have previously reported that the peroxisomal beta-oxidation system for very long chain fatty acids is defective in X-linked childhood adrenoleukodystrophy [(1984) Proc. Natl. Acad. Sci. USA 81, 4203-4207]. In order to elucidate the specific enzyme defect, we examined the oxidation of [1-14C]lignoceric acid, [1-14C]lignoceroyl-CoA and (1-14C)-labelled alpha,beta-unsaturated lignoceroyl-CoA (substrates for the 1st, 2nd, and 3rd steps of the beta-oxidation cycle, respectively). These studies suggest that the pathognomonic accumulation of very long chain fatty acids in X-linked childhood ALD may be due to the defective activity of peroxisomal very long chain (lignoceroyl-CoA) acyl-CoA ligase.

• Authors: M Hashmi, W Stanley, I Singh
• Journal: FEBS letters (FEBS Lett) Vol. 196 Issue 2 Pg. 247-50 (Feb 17 1986) ISSN: 0014-5793 [Print] England
• PMID: 3948997 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, Non-P.H.S., Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Fatty Acids
• Coenzyme A Ligases
• lignoceroyl-CoA ligase

Topics

• Adrenoleukodystrophy (genetics, metabolism)
• Cell Line
• Child
• Coenzyme A Ligases (genetics)
• Diffuse Cerebral Sclerosis of Schilder (metabolism)
• Fatty Acids (metabolism)
• Genetic Linkage
• Humans
• Microbodies (metabolism)
• Oxidation-Reduction
• X Chromosome