Ramsey R B, Banik N L, Davison A N
J Neurol Sci. 1977 May;32(1):69-77. doi: 10.1016/0022-510x(77)90040-5.
The non-hydroxy and hydroxy fatty acids of the major brain galactosphingolipids, cerebroside and sulphatide, have been isolated from white matter, gray matter and myelin of 2 children with adrenoleukodystrophy and from a corresponding control. In addition, cerebroside fatty acids were recovered from a myelin-related fraction on 1 patient. In comparison to control observations, myelin and the myelin-related fraction cerebroside demonstrate a loss of C24:1 and C24h:o fatty acids from normal and hydroxy fatty acid fractions, respectively. White and gray matter galactolipids isolated from 1 patient indicated a signficant increase in short chain (C16, C18 and C18:1) non-hydroxy fatty acids. In the isolated diseased myelin, the ratio of cerebroside hydroxy to non-hydroxy fatty acids was elevated about 1.5 times above the value for normal myelin, whereas the ratio derived for the myelin-related fraction was about two-thirds the values for the control myelin cerebroside.
已从2例肾上腺脑白质营养不良患儿的白质、灰质和髓鞘以及相应对照中分离出主要脑半乳糖鞘脂、脑苷脂和硫脂的非羟基脂肪酸和羟基脂肪酸。此外，还从1例患者的髓鞘相关组分中回收了脑苷脂脂肪酸。与对照观察结果相比，髓鞘和髓鞘相关组分脑苷脂分别显示正常脂肪酸组分和羟基脂肪酸组分中C24:1和C24h:o脂肪酸的缺失。从1例患者分离出的白质和灰质半乳糖脂表明短链（C16、C18和C18:1）非羟基脂肪酸显著增加。在分离出的患病髓鞘中，脑苷脂羟基脂肪酸与非羟基脂肪酸的比例比正常髓鞘的值高出约1.5倍，而髓鞘相关组分的比例约为对照髓鞘脑苷脂值的三分之二。
