肾上腺脑白质营养不良患者脑白质的髓磷脂膜——生化特性

Myelin membrane from adrenoleukodystrophy brain white matter--biochemical properties.

作者信息

Brown F R, Chen W W, Kirschner D A, Frayer K L, Powers J M, Moser A B, Moser H W

出版信息

J Neurochem. 1983 Aug;41(2):341-8. doi: 10.1111/j.1471-4159.1983.tb04748.x.

DOI:10.1111/j.1471-4159.1983.tb04748.x

Abstract

Adrenoleukodystrophy (ALD) is an X-linked progressive neurological disorder characterized by the accumulation of saturated very-long-chain fatty acids (C24 to C30) in lipids, especially cholesterol esters of the brain white matter and adrenal cortex. In the present study we have investigated the localization of accumulated cholesterol esters in brain white matter. During isolation of purified myelin membrane from regions of active demyelination, significant enrichment in cholesterol ester was found in two fractions, mainly in a low-density floating fraction and to a lesser degree in the purified myelin preparation. The fatty acid composition of cholesterol esters from both the ALD floating and myelin fractions was enriched approximately 10-fold in saturated very-long-chain fatty acids (greater than or equal to C24) compared with control preparations.

摘要

肾上腺脑白质营养不良（ALD）是一种X连锁的进行性神经疾病，其特征是饱和超长链脂肪酸（C24至C30）在脂质中蓄积，尤其是在脑白质和肾上腺皮质的胆固醇酯中。在本研究中，我们研究了脑白质中蓄积的胆固醇酯的定位。在从活跃脱髓鞘区域分离纯化髓鞘膜的过程中，发现两个组分中胆固醇酯显著富集，主要在低密度漂浮组分中，在纯化的髓鞘制剂中程度较轻。与对照制剂相比，来自ALD漂浮组分和髓鞘组分的胆固醇酯的脂肪酸组成中饱和超长链脂肪酸（大于或等于C24）富集约10倍。