von-Hippel Lindau and Hypoxia-Inducible Factor at the Center of Renal Cell Carcinoma Biology.
Belzutifan
Hypoxia-inducible factor
Kidney cancer
Renal cell carcinoma
von Hippel-Lindau disease
Journal
Hematology/oncology clinics of North America
ISSN: 1558-1977
Titre abrégé: Hematol Oncol Clin North Am
Pays: United States
ID NLM: 8709473
Informations de publication
Date de publication:
10 2023
10 2023
Historique:
medline:
18
9
2023
pubmed:
4
6
2023
entrez:
3
6
2023
Statut:
ppublish
Résumé
The most common form of kidney cancer is clear cell renal cell carcinoma (ccRCC). Biallelic VHL tumor suppressor gene inactivation is the usual initiating event in both hereditary (VHL Disease) and sporadic ccRCCs. The VHL protein, pVHL, earmarks the alpha subunits of the HIF transcription factor for destruction in an oxygen-dependent manner. Deregulation of HIF2 drives ccRCC pathogenesis. Drugs inhibiting the HIF2-responsive growth factor VEGF are now mainstays of ccRCC treatment. A first-in-class allosteric HIF2 inhibitor was recently approved for treating VHL Disease-associated neoplasms and appears active against sporadic ccRCC in early clinical trials.
Identifiants
pubmed: 37270382
pii: S0889-8588(23)00045-X
doi: 10.1016/j.hoc.2023.04.011
pii:
doi:
Substances chimiques
Von Hippel-Lindau Tumor Suppressor Protein
EC 2.3.2.27
Transcription Factors
0
Types de publication
Journal Article
Review
Research Support, Non-U.S. Gov't
Research Support, N.I.H., Extramural
Langues
eng
Sous-ensembles de citation
IM
Pagination
809-825Informations de copyright
Copyright © 2023 Elsevier Inc. All rights reserved.