Abstract
Germ line inactivation of the von-Hippel-Lindau (VHL) tumor suppressor gene causes von Hippel-Lindau hereditary cancer syndrome, and somatic mutations of this gene have been linked to the development of sporadic hemangioblastomas and clear cell renal carcinomas. The protein encoded by VHL, pVHL, has no known enzymatic activities but interacts with various partner proteins. In this review, various pVHL functions are highlighted. pVHL acts as a multi-purpose adaptor protein that controls different gene expression programs. Through its oxygen-dependent regulation of hypoxia-inducible factor alpha (HIFalpha), pVHL plays a central role in the oxygen-sensing pathway. In addition, many HIFalpha-independent functions of pVHL have recently been identified. These include microtubule-based processes, extracellular matrix assembly and suppression of kidney cyst formation. These complex pVHL functions can explain the diverse consequences of pVHL dysregulation in tumor formation and progression.
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