TY - JOUR
T1 - Adrenoleukodystrophy
T2 - Oleic acid lowers fibroblast saturated C22–26 fatty acids
AU - Rizzo, William B.
AU - Watkins, Paul A.
AU - Phillips, Mary W.
AU - Cranin, Debra
AU - Campbell, Brian
AU - Avigan, Joel
PY - 1986/3
Y1 - 1986/3
N2 - Monounsaturated fatty acids, especially oleic acid (C18:1), decreased the content of saturated very-long-chain fatty acids (VLFA) in cultured skin fibroblasts from patients with adrenoleukodystrophy (ALD) and controls. When confluent ALD fibroblasts were incubated with oleic acid for 5 days in lipid-free medium, which eliminates uptake of exogenous VLFA, the mean cell content of C26:0 was decreased by 33.7 ± 10.1%. Oleic acid inhibited C26:0 synthesis in ALD fibroblasts by 58% and total fatty acid synthesis by 68 to 78%. Therefore, the elevated C26:0 levels in ALD cells may be lowered by inhibiting fatty acid biosynthesis, and inhibition of saturated VLFA synthesis by oleate may be useful in treating ALD.
AB - Monounsaturated fatty acids, especially oleic acid (C18:1), decreased the content of saturated very-long-chain fatty acids (VLFA) in cultured skin fibroblasts from patients with adrenoleukodystrophy (ALD) and controls. When confluent ALD fibroblasts were incubated with oleic acid for 5 days in lipid-free medium, which eliminates uptake of exogenous VLFA, the mean cell content of C26:0 was decreased by 33.7 ± 10.1%. Oleic acid inhibited C26:0 synthesis in ALD fibroblasts by 58% and total fatty acid synthesis by 68 to 78%. Therefore, the elevated C26:0 levels in ALD cells may be lowered by inhibiting fatty acid biosynthesis, and inhibition of saturated VLFA synthesis by oleate may be useful in treating ALD.
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U2 - 10.1212/wnl.36.3.357
DO - 10.1212/wnl.36.3.357
M3 - Article
C2 - 3951702
AN - SCOPUS:0022646258
SN - 0028-3878
VL - 36
SP - 357
EP - 361
JO - Neurology
JF - Neurology
IS - 3
ER -