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

Roy Elias; Qing Zhang; James Brugarolas

doi:10.1097/PPO.0000000000000480

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

Roy Elias, Qing Zhang, James Brugarolas

Research output: Contribution to journal › Review article › peer-review

5 Scopus citations

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 language	English (US)
Pages (from-to)	390-398
Number of pages	9
Journal	Cancer Journal
Volume	26
Issue number	5
DOIs	https://doi.org/10.1097/PPO.0000000000000480
State	Published - 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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10.1097/PPO.0000000000000480

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title = "The von hippel-lindau tumor suppressor gene: Implications and therapeutic opportunities",

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.",

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AU - Elias, Roy

AU - Zhang, Qing

AU - Brugarolas, James

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Y1 - 2020/9/1

N2 - 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.

AB - 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.

KW - AROHIF2

KW - MK-6482

KW - PT2385

KW - PT2977

KW - hypoxia-inducible factor

KW - siRNA

KW - synthetic lethality

KW - tumor evolution

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M3 - Review article

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SN - 1528-9117

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SP - 390

EP - 398

JO - Cancer Journal

JF - Cancer Journal

IS - 5

ER -

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

Abstract

Keywords

ASJC Scopus subject areas

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