Moser H W, Moser A B
Kennedy Krieger Institute, Johns Hopkins University, Baltimore, Maryland 21205, USA.
Lipids. 1996 Mar;31 Suppl:S141-4. doi: 10.1007/BF02637066.
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.
超长链脂肪酸（VLCFA）水平异常升高是目前已确定的15种过氧化物酶体疾病中9种疾病的特征。饱和VLCFA在X连锁肾上腺脑白质营养不良中蓄积，似乎会破坏膜结构，并可能在脑部炎症反应的发病机制中起作用。在患者仍无症状时开始饮食治疗可能具有临床益处。
