Alpha-oxidation of 3-methyl-substituted fatty acids and its thiamine dependence

被引：33

作者：

Casteels, M ^{[1
]}

Foulon, V ^{[1
]}

Mannaerts, GP ^{[1
]}

Van Veldhoven, PP ^{[1
]}

机构：

[1] Katholieke Univ Leuven, Afdeling Farmacol, Dept Mol Cell Biol, B-3000 Louvain, Belgium

来源：

EUROPEAN JOURNAL OF BIOCHEMISTRY | 2003年 / 270卷 / 08期

关键词：

alpha-oxidation; thiamine pyrophosphate; peroxisomes; lyase; Adult Refsum Disease;

D O I：

10.1046/j.1432-1033.2003.03534.x

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

3-Methyl-branched fatty acids, as phytanic acid, undergo peroxisomal alpha-oxidation in which they are shortened by 1 carbon atom. This process includes four steps: activation, 2-hydroxylation, thiamine pyrophosphate dependent cleavage and aldehyde dehydrogenation. The thiamine pyrophosphate dependence of the third step is unique in peroxisomal mammalian enzymology. Human pathology due to a deficient alpha-oxidation is mostly linked to mutations in the gene coding for the second enzyme of the sequence, phytanoyl-CoA hydroxylase.

引用

页码：1619 / 1627

页数：9

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