The von Hippel-Lindau Tumor Suppressor Gene
Roy Elias, Qing Zhang, James Brugarolas
Abstract
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.
Topics & Concepts
Tumor suppressor geneSuppressorCancer researchGeneMedicineBiologyGeneticsCarcinogenesisEpigenetics and DNA MethylationRenal cell carcinoma treatmentCancer, Hypoxia, and Metabolism