Kaelin W G, Maher E R
Division of Adult Oncology, Dana-Farber Cancer Hospital, Boston, MA, USA.
Trends Genet. 1998 Oct;14(10):423-6. doi: 10.1016/s0168-9525(98)01558-3.
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.
VHL肿瘤抑制基因（TSG）在调节人类肾细胞的生长和分化中起关键的“守门人”作用，VHL基因的失活是人类肾癌中最常见的遗传事件。VHL与视网膜母细胞瘤TSG的遗传学有许多相似之处，但VHL肿瘤发生模型更为复杂。在这里，我们研究了VHL TSG遗传学和功能方面的现有知识，并强调了VHL基因如何提供一个范例来说明TSG生物学的许多方面。
