Wilson R, Tocher D R, Sargent J R
Department of Biological and Molecular Sciences, School of Natural Sciences, University of Stirling, Scotland, UK.
J Neurol Sci. 1992 Jun;109(2):207-14. doi: 10.1016/0022-510x(92)90170-p.
The conversion of [1-14C]16:0 to very-long-chain saturated fatty acids (VLCSFA) was greater in fibroblasts from adrenoleukodystrophy (ALD) patients than fibroblasts from normal subjects. Added 23:1(n-9) decreased the formation of VLCSFA from [1-14C]16:0 in ALD fibroblasts to the value found in normal fibroblasts. Chain-elongation as well as extensive chain-shortening of added 20:1(n-9), 22:1(n-9), 23:1(n-9) and 24:1(n-9) occurred in both normal and ALD fibroblasts, with chain-shortening being less in ALD than in normal fibroblasts. Added 18:1(n-9) together with 22:1(n-9) reduced the levels of both VLCSFA and total n-6 and n-3 polyunsaturated fatty acids (PUFA) in normal and ALD fibroblasts. The levels of total (n-6) and (n-3) PUFA but not the levels of VLCSFA were readily restored by culturing the cells in the presence of 18:1(n-9), 22:1(n-9), 18:2(n-6) and 18:3(n-3). The results are consistent with added monounsaturated fatty acids reducing levels of VLCSFA in ALD fibroblasts by depressing their biosynthesis from 16:0. They also support the use of oils rich in long chain monoenes as a dietary therapy for ALD patients but caution that the PUFA status of ALD patients should be monitored with a view to dietary supplementation, if necessary, with PUFA.
肾上腺脑白质营养不良（ALD）患者的成纤维细胞中，[1-14C]16:0向极长链饱和脂肪酸（VLCSFA）的转化比正常受试者的成纤维细胞更显著。添加23:1(n-9)可使ALD成纤维细胞中由[1-14C]16:0生成VLCSFA的量降至正常成纤维细胞中的水平。在正常和ALD成纤维细胞中，添加的20:1(n-9)、22:1(n-9)、23:1(n-9)和24:1(n-9)均发生链延长以及广泛的链缩短，且ALD成纤维细胞中的链缩短程度低于正常成纤维细胞。添加18:1(n-9)与22:1(n-9)可降低正常和ALD成纤维细胞中VLCSFA以及总n-6和n-3多不饱和脂肪酸（PUFA）的水平。通过在18:1(n-9)、22:1(n-9)、18:2(n-6)和18:3(n-3)存在的情况下培养细胞，总（n-6）和（n-3）PUFA的水平可轻易恢复，但VLCSFA的水平未恢复。这些结果与添加的单不饱和脂肪酸通过抑制VLCSFA从16:0的生物合成来降低ALD成纤维细胞中VLCSFA水平一致。它们还支持使用富含长链单烯的油作为ALD患者的饮食疗法，但提醒应监测ALD患者的PUFA状态，必要时进行PUFA饮食补充。
