The VHL tumour-suppressor gene paradigm

WG Kaelin, ER Maher - Trends in genetics, 1998 - cell.com
WG Kaelin, ER Maher
Trends in genetics, 1998cell.com
The VHL tumour-suppressor gene (TSG) has a criticalgatekeeper'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 …
Abstract
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.
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