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The von Hippel-Lindau Tumor Suppressor Gene: Implications and Therapeutic Opportunities.
The Cancer Journal ( IF 2.6 ) Pub Date : 2020-09-01 , DOI: 10.1097/ppo.0000000000000480
Roy Elias 1 , Qing Zhang 2 , James Brugarolas 1
Affiliation  

The discovery of the von Hippel-Lindau (VHL) gene marked a milestone in our understanding of clear cell renal cell carcinoma (ccRCC) pathogenesis. VHL inactivation is not only a defining feature of ccRCC, but also the initiating event. Herein, we discuss canonical and noncanonical pVHL functions, as well as breakthroughs shaping our understanding of ccRCC evolution and evolutionary subtypes. We conclude by presenting evolving strategies to therapeutically exploit effector mechanisms downstream of pVHL.



中文翻译:

von Hippel-Lindau 肿瘤抑制基因:意义和治疗机会。

von Hippel-Lindau ( VHL ) 基因的发现标志着我们理解透明细胞肾细胞癌 (ccRCC) 发病机制的一个里程碑。VHL失活不仅是 ccRCC 的定义特征,而且还是起始事件。在此,我们讨论规范和非规范 pVHL 功能,以及塑造我们对 ccRCC 进化和进化亚型理解的突破。最后,我们提出了在治疗上利用 pVHL 下游效应机制的进化策略。

更新日期:2020-09-21
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