Plasma phospholipid fatty acids in X-linked adrenoleukodystrophy.
Plasma phospholipid fatty acids in X-linked adrenoleukodystrophy.
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X连锁肾上腺脑白质营养不良中的血浆磷脂脂肪酸。
DOI：
10.1093/clinchem/42.3.454
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发表时间：
1996
影响因子：
9.3
通讯作者：
D. Donnari
中科院分区：
文献类型：
--
作者：
M. Aveldaño;D. Donnari
对一例X连锁肾上腺脑白质营养不良患儿进行了血细胞和血浆脂质分类及其脂肪酸分析。这种疾病的典型特征是具有很长链的饱和脂肪酸增加，几乎完全发生在鞘磷脂中。在该油脂中，木油酸(24：0)和十六烷酸(26：0)的比例增加，而18：0、20：0和24：1的比例下降。在其余的脂肪类中，尤其是在胆固醇酯和三酰甘油中，亚油酸(18：2)的比例下降，而油酸(18：1)的比例增加。在甘油磷脂中，20：4n-6、22：5n-6和22：6n-3等多不饱和脂肪酸减少，而其直接前体20：3n-6、22：4n-6和22：5n-3相对增加，表明脂肪酸的去饱和机制存在缺陷。虽然不那么明显，但在患者的母亲身上也看到了类似的变化趋势；在两种情况下，所有的变化在血清中都比在血细胞中更明显。
Blood cell and plasma lipid classes and their fatty acids were analyzed in a child with X-linked adrenoleukodystrophy. The increase in saturated fatty acids with very long chains typical of this disease occurred almost exclusively in sphingomyelin. In this lipid, the proportion of lignoceric (24:0) and hexacosanoic (26:0) acids increased while that of 18:0, 20:0, and 24:1 decreased. In the rest of the lipid classes, but especially in cholesteryl esters and triacylglycerols, the proportion of linoleate (18:2) decreased while that of oleate (18:1) increased. In glycerophospholipids, polyunsaturated fatty acids such as 20:4n-6, 22:5n-6, and 22:6n-3 were reduced while their immediate precursors, 20:3n-6, 22:4n-6, and 22:5n-3, respectively, were relatively increased, suggesting a defect in fatty acid desaturation mechanisms. Although less pronounced, a similar trend of changes was seen in the patient's mother; in both, all alterations were more marked in serum than in blood cells.
DOI：
10.1016/s0021-9258(18)54882-1
发表时间：
1991-10
期刊：
The Journal of biological chemistry
影响因子：
--
作者：
A. Voss;M. Reinhart;S. Sankarappa;H. Sprecher
通讯作者：
A. Voss;M. Reinhart;S. Sankarappa;H. Sprecher
影响因子：
6.5
作者：
W. R. Morrison;L. Smith
通讯作者：
W. R. Morrison;L. Smith