Effects of exogenous monounsaturated fatty acids on fatty acid metabolism in cultured skin fibroblasts from adrenoleukodystrophy patients

Citation

Wilson R, Tocher DR & Sargent JR (1992) Effects of exogenous monounsaturated fatty acids on fatty acid metabolism in cultured skin fibroblasts from adrenoleukodystrophy patients. Journal of the Neurological Sciences, 109 (2), pp. 207-214. https://doi.org/10.1016/0022-510X%2892%2990170-P

Abstract
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 normalfibroblasts. 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.

Keywords
ADRENOLEUKODYSTROPHY; HEXACOSANOIC ACID; VERY LONG CHAIN SATURATED FATTY ACIDS

Journal
Journal of the Neurological Sciences: Volume 109, Issue 2