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脂肪酸和代谢产物穿过过氧化物酶体膜的运输。

Transport of fatty acids and metabolites across the peroxisomal membrane.

作者信息

Hettema E H, Tabak H F

机构信息

Department of Biochemistry, Academic Medical Center, Amsterdam, The Netherlands.

出版信息

Biochim Biophys Acta. 2000 Jun 26;1486(1):18-27. doi: 10.1016/s1388-1981(00)00045-7.

Abstract

The peroxisomal membrane forms a permeability barrier for a wide variety of metabolites required for and formed during fatty acid beta-oxidation. To communicate with the cytoplasm and mitochondria, peroxisomes need dedicated proteins to transport such hydrophilic molecules across their membranes. Genetic and biochemical studies in the yeast Saccharomyces cerevisiae have identified enzymes for redox shuttles as well as the first peroxisomal membrane transporter. This peroxisomal ATP-binding cassette transporter (Pat) is highly homologous to the gene mutated in X-linked adrenoleukodystrophy (X-ALD). The yeast Pat is required for import of activated fatty acids into peroxisomes suggesting that this is the primary defect in X-ALD.

摘要

过氧化物酶体膜对脂肪酸β-氧化所需及过程中形成的多种代谢物形成渗透屏障。为了与细胞质和线粒体进行通讯,过氧化物酶体需要特定的蛋白质来跨膜转运此类亲水分子。对酿酒酵母进行的遗传学和生物化学研究已鉴定出氧化还原穿梭酶以及首个过氧化物酶体膜转运蛋白。这种过氧化物酶体ATP结合盒转运蛋白(Pat)与X连锁肾上腺脑白质营养不良(X-ALD)中发生突变的基因高度同源。酵母Pat是将活化脂肪酸导入过氧化物酶体所必需的,这表明这是X-ALD中的主要缺陷。

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