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

Hugo W. Moser, Ann B. Moser

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
JournalLipids
Volume31
Issue number1
DOIs
StatePublished - Mar 1996

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Peroxisomal Disorders
very long chain fatty acids
Fatty Acids
pathogenesis
Adrenoleukodystrophy
therapeutics
diet therapy
Membrane structures
Brain
inflammation
brain
Membranes
Therapeutics

ASJC Scopus subject areas

  • Medicine (miscellaneous)
  • Biochemistry
  • Biochemistry, Genetics and Molecular Biology(all)
  • Food Science

Cite this

Very long-chain fatty acids in diagnosis, pathogenesis, and therapy of peroxisomal disorders. / Moser, Hugo W.; Moser, Ann B.

In: Lipids, Vol. 31, No. 1, 03.1996.

Research output: Contribution to journalArticle

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