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

FEBS Lett. 1986 Feb 17;196(2):247-50. doi: 10.1016/0014-5793(86)80256-3.


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.

Publication types

  • 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.

MeSH terms

  • Adrenoleukodystrophy / genetics
  • Adrenoleukodystrophy / 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


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