Antoku Y, Sakai T, Goto I, Miyoshino S, Iwashita H, Kuroiwa Y
Neurology. 1985 Oct;35(10):1512-4. doi: 10.1212/wnl.35.10.1512.
"Tightly bound" and "loosely bound" fatty acids in erythrocyte membranes were analyzed in three patients with adrenoleukodystrophy, three probable carriers, and eight controls. The ratios of C28:0 or C26:0 to C22:0 or C20:0 in the tightly bound fatty acids of three patients were significantly higher than those of controls, and the ratios in two of three probable carriers were higher than those in controls. The ratios of C26:0 to C22:0 or C20:0 in the loosely bound fatty acids of three patients and three probable carriers were also significantly higher than those of controls. Since tightly bound fatty acids in membrane proteins are found not only in erythrocyte membranes but also in myelin proteins, the abnormality of tightly bound fatty acids may be related to the demyelination in adrenoleukodystrophy.
对三名肾上腺脑白质营养不良患者、三名可能的携带者和八名对照者的红细胞膜中“紧密结合”和“松散结合”的脂肪酸进行了分析。三名患者紧密结合脂肪酸中C28:0或C26:0与C22:0或C20:0的比值显著高于对照者，三名可能携带者中的两名的该比值高于对照者。三名患者和三名可能携带者松散结合脂肪酸中C26:0与C22:0或C20:0的比值也显著高于对照者。由于膜蛋白中紧密结合的脂肪酸不仅存在于红细胞膜中，也存在于髓磷脂蛋白中，紧密结合脂肪酸的异常可能与肾上腺脑白质营养不良中的脱髓鞘有关。
