Steroidogenic acute regulatory (StAR) protein: what's new?
Steroidogenic acute regulatory (StAR) protein: what's new?
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DOI：
10.1002/(sici)1521-1878(199909)21:9
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发表时间：
1999-09
期刊：
影响因子：
4
通讯作者：
D. Stocco
中科院分区：
文献类型：
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作者：
D. Stocco
为了响应类固醇生成肾上腺和性腺细胞的营养激素刺激，类固醇激素的急性生物合成发生在几分钟到几十分钟的数量级内，并且可以与发生在数小时数量级上的慢性调节形成对比。类固醇生成急性调节 (StAR) 蛋白是急性调节阶段不可或缺的组成部分，其功能是通过快速介导所有类固醇激素的底物胆固醇从线粒体外膜转移到线粒体内膜，在线粒体内膜中被裂解为孕烯醇酮（第一种形成的类固醇）。胆固醇的这种转移构成了类固醇生成的限速步骤。为了强调其重要性，StAR 基因的突变已被证明是潜在致命疾病类脂性先天性肾上腺增生的唯一原因，在这种疾病中，受影响的个体几乎不合成类固醇。自 1994 年克隆小鼠 cDNA 以来，许多观察结果证实了 StAR 在调节类固醇生成中的关键作用。本综述的目的是简要总结 StAR 的一些背景材料，然后尝试更新有关 StAR 蛋白的一些最新有趣的发现。 BioEssays 21：768–775，1999 年。© 1999 John Wiley & Sons, Inc.
In response to trophic hormone stimulation of steroidogenic adrenal and gonadal cells, the acute biosynthesis of steroid hormones occurs in the order of minutes to tens of minutes and can be contrasted to chronic regulation, which occurs on the order of hours. The steroidogenic acute regulatory (StAR) protein is an indispensable component in the acute regulatory phase and functions by rapidly mediating the transfer of the substrate for all steroid hormones, cholesterol, from the outer to the inner mitochondrial membrane where it is cleaved to pregnenolone, the first steroid formed. This transfer of cholesterol constitutes the rate‐limiting step in steroidogenesis. To underscore its importance, mutations in the StAR gene have been shown to be the only cause of the potentially fatal disease lipoid congenital adrenal hyperplasia, in which affected individuals synthesize virtually no steroids. Since the cloning of the murine cDNA in 1994, many observations have substantiated the critical role of StAR in regulated steroidogenesis. The purpose of this review will be to summarize briefly some background material on StAR and then attempt to update several recent and interesting findings on the StAR protein. BioEssays 21:768–775, 1999. © 1999 John Wiley & Sons, Inc.