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Molecular basis of the VHL hereditary cancer syndrome
Author(s): Kaelin WG
Source: NATURE REVIEWS CANCER    Volume: 2    Issue: 9    Pages: 673-682    Published: SEP 2002  
Times Cited: 275     References: 144     
Abstract: 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.
Document Type: Review
Language: English
Reprint Address: Kaelin, WG (reprint author), Harvard Univ, Sch Med, Howard Hughes Med Inst, Boston, MA 02115 USA
Addresses:
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
Publisher: NATURE PUBLISHING GROUP, MACMILLAN BUILDING, 4 CRINAN ST, LONDON N1 9XW, ENGLAND
Subject Category: Oncology
IDS Number: 636GZ
ISSN: 1474-175X
DOI: 10.1038/nrc885
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