PHYSIOLOGICAL SIGNIFICANCE OF FATTY-ACID ELONGATION SYSTEM IN ADRENOLEUKODYSTROPHY

被引：29

作者：

KOIKE, R ^{[1
]}

TSUJI, S ^{[1
]}

OHNO, T ^{[1
]}

SUZUKI, Y ^{[1
]}

ORII, T ^{[1
]}

MIYATAKE, T ^{[1
]}

机构：

[1] GIFU UNIV,DEPT PEDIAT,GIFU 500,JAPAN

来源：

JOURNAL OF THE NEUROLOGICAL SCIENCES | 1991年 / 103卷 / 02期

关键词：

ADRENOLEUKODYSTROPHY; VERY LONG CHAIN FATTY ACIDS; FATTY ACID METABOLISM; UNSATURATED FATTY ACIDS; DIET THERAPY; FATTY ACID ELONGATION SYSTEM;

D O I：

10.1016/0022-510X(91)90163-2

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

We studied the metabolism of radioactive fatty acids in cultured skin fibroblasts from patients with adrenoleukodystrophy (ALD) and Zellweger syndrome (ZS) to clarify the physiological significance of the fatty acid elongation system in the accumulation of very long chain fatty acids (VLFA) in ALD. A substantial amount of radioactive C26:0 was synthesized from [18-C-14]stearic acid by ALD and ZS fibroblasts, whereas radioactive C26:0 was not detected in control fibroblasts. Kinetic studies demonstrated that the production of radioactive C24:0 and C26:0 in ALD fibroblasts is higher than that of ZS fibroblasts, which suggests that the fatty acid elongation activity plays an important role for the accumulation of VLFA in ALD, in addition to the decreased VLFA oxidation activities. The addition of monounsaturated fatty acids including oleic acid and erucic acid specifically lowered the formation rate of VLFA without any significant effect on degradation activities of VLFA both in ALD and control fibroblasts. The results suggest that the mechanism of decrease of VLFA by administration of monounsaturated fatty acids is based on the inhibition of fatty acid elongation activity.

引用

页码：188 / 194

页数：7

共 29 条

[1] CHARACTERIZATION OF RAT-BRAIN MICROSOMAL ACYL-COENZYME-A LIGASES - DIFFERENT ENZYMES FOR THE SYNTHESIS OF PALMITOYL-COENZYME-A AND LIGNOCEROYL-COENZYME-A [J].