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| Molecular basis of the VHL hereditary cancer syndrome |
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| 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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