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Trends Genet. 1998 Oct;14(10):423-6.

The VHL tumour-suppressor gene paradigm.

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Division of Adult Oncology, Dana-Farber Cancer Hospital, Boston, MA, USA.


The VHL tumour-suppressor gene (TSG) has a critical 'gatekeeper' role in regulating growth and differentiation of human kidney cells, and inactivation of the VHL gene is the most frequent genetic event in human kidney cancer. There are many similarities between the genetics of the VHL and retinoblastoma TSGs, but the VHL tumourigenesis model is more complex. Here, we examine the current knowledge of the genetics and functional aspects of the VHL TSG, and emphasize how the VHL gene provides a paradigm that illustrates many aspects of TSG biology.

[Indexed for MEDLINE]

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