Farzan Shohreh F, Singh Samer, Schilling Neal S, Robbins David J
Department of Pharmacology and Toxicology, Dartmouth Medical School, Hanover, NH 03755, USA.
Am J Physiol Gastrointest Liver Physiol. 2008 Apr;294(4):G844-9. doi: 10.1152/ajpgi.00564.2007. Epub 2008 Jan 31.
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.
分泌蛋白的刺猬（Hh）家族对于胃肠道的发育和维持至关重要。Hh被认为具有形态发生素、促有丝分裂原、细胞存活因子和轴突导向因子的功能。鉴于其在发育以及多种疾病状态中的广泛作用，了解Hh功能和活性的调节至关重要。然而，Hh信号传导的研究因其独特的生物学特性而受到阻碍。Hh的独特之处在于它是唯一一种被胆固醇共价修饰的蛋白质，而胆固醇又反过来影响其定位、释放、移动和活性的许多方面。在考虑Hh的生理作用时，所有这些都是重要因素，并且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。本综述旨在全面概述Hh的生物合成和移动如何促进其生物活性。
