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标题：Identification of the peroxisomal β-oxidation enzymes involved in the biosynthesis of docosahexaenoic acid
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作者：Sacha Ferdinandusse ; Simone Denis ; Petra A. W. Mooijer 等
期刊名称：JLR Papers In Press
印刷版ISSN：0022-2275
电子版ISSN：1539-7262
出版年度：2001
卷号：42
期号：12
页码：1987-1995
语种：English
出版社：American Society for Biochemistry and Molecular Biology
摘要：DHA (C22:6n-3) is an important PUFA implicated in a number of (patho)physiological processes. For a long time, the exact mechanism of DHA formation has remained unclear, but now it is known that it involves the production of tetracosahexaenoic acid (C24:6n-3) from dietary linolenic acid (C18:3n-3) via a series of elongation and desaturation reactions, followed by β-oxidation of C24:6n-3 to C22:6n-3. Although DHA is deficient in patients lacking peroxisomes, the intracellular site of retroconversion of C24:6n-3 has remained controversial. By making use of fibroblasts from patients with defined mitochondrial and peroxisomal fatty acid oxidation defects, we show in this article that peroxisomes, and not mitochondria, are involved in DHA formation by catalyzing the β-oxidation of C24:6n-3 to C22:6n-3. Additional studies of fibroblasts from patients with X-linked adrenoleukodystrophy, straight-chain acyl-CoA oxidase (SCOX) deficiency, d -bifunctional protein (DBP) deficiency, and rhizomelic chondrodysplasia punctata type 1, and of fibroblasts from l -bifunctional protein and sterol carrier protein X (SCPx) knockout mice, show that the main enzymes involved in β-oxidation of C24:6n-3 to C22:6n-3 are SCOX, DBP, and both 3-ketoacyl-CoA thiolase and SCPx. These findings are of importance for the treatment of patients with a defect in peroxisomal β-oxidation. —Ferdinandusse, S., S. Denis, P. A. W. Mooijer, Z. Zhang, J. K. Reddy, A. A. Spector, and R. J. A. Wanders. Identification of the peroxisomal β-oxidation enzymes involved in the biosynthesis of docosahexaenoic acid. J. Lipid Res. 2001. 42: 1987–1995.
关键词：d -bifunctional protein ; 3-ketoacyl-CoA thiolase ; linolenic acid ; polyunsaturated fatty acids ; straight-chain acyl-CoA oxidase ; tetracosahexaenoic acid ; peroxisomal and mitochondrial fatty acid oxidation disorders ; sterol carrier protein X