Very long-chain fatty acids in diagnosis, pathogenesis, and therapy of peroxisomal disorders

Lipids. 1996 Mar:31 Suppl:S141-4. doi: 10.1007/BF02637066.

Abstract

Abnormally high levels of very long-chain fatty acids (VLCFA) are a feature in nine of the fifteen peroxisomal disorders that have been identified so far. Saturated VLCFA accumulate in X-linked adrenoleukodystrophy, appear to disrupt membrane structure, and may play a role in the pathogenesis of a brain inflammatory response. Dietary therapy initiated when patients are still asymptomatic may be of clinical benefit.

Publication types

  • Review

MeSH terms

  • Adrenoleukodystrophy / genetics
  • Adrenoleukodystrophy / metabolism
  • Fatty Acids / metabolism*
  • Genetic Linkage
  • Humans
  • Peroxisomal Disorders / diagnosis
  • Peroxisomal Disorders / etiology
  • Peroxisomal Disorders / metabolism*
  • Peroxisomal Disorders / therapy
  • X Chromosome
  • Zellweger Syndrome / blood

Substances

  • Fatty Acids