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 language||English (US)|
|Issue number||3 SUPPL.|
|State||Published - Mar 1 1996|
ASJC Scopus subject areas
- Organic Chemistry
- Cell Biology