The adventures of sonic hedgehog in development and repair. III. Hedgehog processing and biological activity
The adventures of sonic hedgehog in development and repair. III. Hedgehog processing and biological activity
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DOI：
10.1152/ajpgi.00564.2007
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发表时间：
2008-04-01
影响因子：
4.5
通讯作者：
Robbins, David J.
中科院分区：
文献类型：
--
作者：
Farzan, Shohreh F.;Singh, Samer;Robbins, David J.
Hedgehog（Hh）分泌蛋白家族对于胃肠道的发育和维持方面是必需的。Hh被认为是一种形态发生素、有丝分裂原、细胞存活因子和轴突导向因子。鉴于其在发育中的广泛作用，以及在各种疾病状态下，了解Hh功能和活性的调节至关重要。然而，Hh信号转导的研究一直受到其不寻常的生物学的阻碍。Hh的独特之处在于它是唯一被胆固醇共价修饰的蛋白质，而胆固醇反过来又影响其定位、释放、运动和活性的许多方面。在考虑Hh的生理作用时，所有这些都是重要的因素，动物已经发展出一个复杂的调节系统，负责促进和抑制Hh的活性。这篇综述旨在对Hh的生物合成和运动如何有助于其生物活性进行广泛的概述。
The Hedgehog (Hh) family of secreted proteins is necessary for aspects of the development and maintenance of the gastrointestinal tract. Hh is thought to function as a morphogen, a mitogen, a cell survival factor, and an axon guidance factor. Given its wide role in development, as well as in a variety of disease states, understanding the regulation of Hh function and activity is critically important. However, the study of Hh signaling has been impeded by its unusual biology. Hh is unique in that it is the only protein covalently modified by cholesterol, which in turn affects numerous aspects of its localization, release, movement, and activity. All are important factors when considering Hh's physiological role, and animals have developed an intricate system of regulators responsible for both promoting and inhibiting the activity of Hh. This review is intended to give a broad overview of how the biosynthesis and movement of Hh contributes to its biological activity.