Molecular basis of the VHL hereditary cancer syndrome

被引：640

作者：

Kaelin, WG ^{[1
]}

机构：

[1] Harvard Univ, Sch Med, Howard Hughes Med Inst, Boston, MA 02115 USA

[2] Harvard Univ, Sch Med, Dana Farber Canc Inst, Boston, MA 02115 USA

[3] Harvard Univ, Brigham & Womens Hosp, Sch Med, Boston, MA 02115 USA

来源：

NATURE REVIEWS CANCER | 2002年 / 2卷 / 09期

关键词：

D O I：

10.1038/nrc885

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

The von Hippel-Lindau hereditary cancer syndrome was first described about 100 years ago, The unusual clinical features of this disorder predicted a role for the von Hippel-Lindau gene (VHL) in the oxygen-sensing pathway. Indeed, recent studies of this gene have helped to decipher how cells sense changes in oxygen availability, and have revealed a previously unappreciated role of prolyl hydroxylation in intracellular signalling. These studies, in turn, are laying the foundation for the treatment of a diverse set of disorders, including cancer, myocardial infarction and stroke.

引用

页码：673 / 682

页数：10

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