The von hippel-lindau tumor suppressor gene: Implications and therapeutic opportunities

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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.

Original languageEnglish (US)
Pages (from-to)390-398
Number of pages9
JournalCancer Journal
Volume26
Issue number5
DOIs
StatePublished - Sep 1 2020

Keywords

  • AROHIF2
  • MK-6482
  • PT2385
  • PT2977
  • hypoxia-inducible factor
  • siRNA
  • synthetic lethality
  • tumor evolution

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

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