β氧化
过氧化物酶体
肾上腺脑白质营养不良
下调和上调
脂肪酸
过氧化物酶体障碍
生物化学
化学
泽尔韦格综合征
氧化还原
生物
基因
作者
Ronald J. A. Wanders,J.C. Komen,Stephan Kemp
出处
期刊:FEBS Journal
[Wiley]
日期:2010-11-09
卷期号:278 (2): 182-194
被引量:201
标识
DOI:10.1111/j.1742-4658.2010.07947.x
摘要
Fatty acids (FAs) can be degraded via different mechanisms including α-, β- and ω-oxidation. In humans, a range of different genetic diseases has been identified in which either mitochondrial FA β-oxidation, peroxisomal FA β-oxidation or FA α-oxidation is impaired. Treatment options for most of these disorders are limited. This has prompted us to study FA ω-oxidation as a rescue pathway for these disorders, based on the notion that if the ω-oxidation of specific FAs could be upregulated one could reduce the accumulation of these FAs and the subsequent detrimental effects in the different groups of disorders. In this minireview, we describe our current state of knowledge in this area with special emphasis on Refsum disease and X-linked adrenoleukodystrophy.
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