Article
Molecular basis of the VHL hereditary cancer syndrome.
Nature reviews. Cancer - 1 Sept 2002
Kaelin William G
Abstract excerpt
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...
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