VERY LONG-CHAIN FATTY-ACIDS IN DIAGNOSIS, PATHOGENESIS, AND THERAPY OF PEROXISOMAL DISORDERS

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