2,6-dimethylheptanoyl-CoA is a specific substrate for long-chain acyl-CoA dehydrogenase (LCAD): evidence for a major role of LCAD in branched-chain fatty acid oxidation

被引：35

作者：

Wanders, RJA

Denis, S

Ruiter, JPN

IJlst, L

Dacremont, G

机构：

[1] Univ Amsterdam, Acad Med Ctr, Emma Childrens Hosp, Dept Clin Chem, NL-1100 DE Amsterdam, Netherlands

[2] Univ Amsterdam, Acad Med Ctr, Emma Childrens Hosp, Dept Pediat, NL-1100 DE Amsterdam, Netherlands

[3] State Univ Ghent, Dept Pediat, B-9000 Gent, Belgium

来源：

BIOCHIMICA ET BIOPHYSICA ACTA-LIPIDS AND LIPID METABOLISM | 1998年 / 1393卷 / 01期

关键词：

mitochondrial fatty acid oxidation; fatty acid; oxidation; inherited disease;

D O I：

10.1016/S0005-2760(98)00053-8

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：

Oxidation of straight-chain fatty acids in mitochondria involves the complicated interaction between a large variety of different enzymes. So far four different mitochondrial straight-chain acyl-CoA dehydrogenases have been identified. The physiological function of three of the four acyl-CoA dehydrogenases has been resolved in recent years especially from studies on patients suffering from certain inborn errors of mitochondrial fatty acid beta-oxidation. The physiological role of long-chain acyl-CoA dehydrogenase (LCAD) has remained obscure, however. The results described in this paper provide strong evidence suggesting that LCAD plays a central role in branched-chain fatty acid metabolism since it turns out to be the major acyl-CoA dehydrogenase reacting with 2,6-dimethylheptanoyl-CoA, a metabolite of pristanic acid, which itself is the alpha-oxidation product of phytanic acid. (C) 1998 Elsevier Science B.V. All rights reserved.

引用

页码：35 / 40

页数：6

共 29 条

[1] Cloning and characterization of human very-long-chain acyl-CoA dehydrogenase cDNA, chromosomal assignment of the gene and identification in four patients of nine different mutations within the VLCAD gene [J].