Avigan J, Campbell B D, Yost D A, Hernell O, Holmgren G, Jagell S F
Neurology. 1985 Mar;35(3):401-3. doi: 10.1212/wnl.35.3.401.
Cultured skin fibroblasts from two patients with Sjögren-Larsson syndrome (SLS) and from a normal control were analyzed for trienoic and tetraenoic fatty acids. In addition, we assayed desaturation of [1-14C]linoleic acid in cells from four patients and four controls. There was no significant effect of the disease on the composition of polyunsaturated fatty acids or on the rate of linoleic acid desaturation in fibroblasts. The results indicate the presence of delta 5- and delta 6-fatty acid desaturases in cells from SLS patients.
对两名舍格伦-拉松综合征（SLS）患者及一名正常对照者的培养皮肤成纤维细胞进行了三烯酸和四烯酸脂肪酸分析。此外，我们检测了4名患者和4名对照者细胞中[1-14C]亚油酸的去饱和作用。该疾病对成纤维细胞中多不饱和脂肪酸的组成或亚油酸去饱和速率没有显著影响。结果表明SLS患者细胞中存在δ5-和δ6-脂肪酸去饱和酶。
