The von Hippel-Lindau Tumor Suppressor Gene: Implications and Therapeutic Opportunities.


Journal

Cancer journal (Sudbury, Mass.)
ISSN: 1540-336X
Titre abrégé: Cancer J
Pays: United States
ID NLM: 100931981

Informations de publication

Date de publication:
Historique:
entrez: 18 9 2020
pubmed: 19 9 2020
medline: 28 9 2021
Statut: ppublish

Résumé

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.

Identifiants

pubmed: 32947307
doi: 10.1097/PPO.0000000000000480
pii: 00130404-202009000-00005
pmc: PMC9812266
mid: NIHMS1848441
doi:

Substances chimiques

Von Hippel-Lindau Tumor Suppressor Protein EC 2.3.2.27
VHL protein, human EC 6.3.2.-

Types de publication

Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, Non-P.H.S. Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

390-398

Subventions

Organisme : NCI NIH HHS
ID : P50 CA196516
Pays : United States
Organisme : NCI NIH HHS
ID : R01 CA211732
Pays : United States

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Auteurs

Roy Elias (R)

From the Kidney Cancer Program, Simmons Comprehensive Cancer Center.

Qing Zhang (Q)

Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX.

James Brugarolas (J)

From the Kidney Cancer Program, Simmons Comprehensive Cancer Center.

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