DECREASED CONTENT OF DOCOSAHEXAENOATE AND ARACHIDONATE IN PLASMA PHOSPHOLIPIDS IN USHERS SYNDROME
DECREASED CONTENT OF DOCOSAHEXAENOATE AND ARACHIDONATE IN PLASMA PHOSPHOLIPIDS IN USHERS SYNDROME
复制标题
DOI：
10.1016/s0006-291x(86)80215-7
复制
发表时间：
1986-12-15
影响因子：
3.1
通讯作者：
PELIAS, MZ
中科院分区：
文献类型：
--
作者：
BAZAN, NG;SCOTT, BL;PELIAS, MZ
二十二碳六烯酸和花生四烯酸被发现显着减少从Usher综合征患者的血浆磷脂。这些患者血浆三酰甘油的脂肪酸含量没有变化。Usher综合征是一种常染色体隐性遗传疾病，涉及遗传性视觉细胞变性。光感受器膜富含二十二碳六烯酸和花生四烯酸，并且可能发生影响这些多不饱和脂肪酸的代谢缺陷。此外，失明可能是由于，至少部分是由于感光膜的不饱和磷脂的改变。
Docosahexaenoate and arachidonate were found to be significantly decreased in plasma phospholipids from Usher's syndrome patients. The fatty acid content of plasma triacylglycerols was not changed in these patients. Usher's syndrome, an autosomal recessive disorder, involves an inherited visual cell degeneration. Photoreceptor membranes are richly endowed with docosahexaenoate and arachidonate, and a metabolic defect affecting these polyunsaturated fatty acids may occur. Moreover, blindness may be due, at least partially, to an alteration in the unsaturated phospholipids of photoreceptor membranes.