Clear cell renal cell carcinoma ontogeny and mechanisms of lethality

E Jonasch, CL Walker, WK Rathmell - Nature Reviews Nephrology, 2021 - nature.com
The molecular features that define clear cell renal cell carcinoma (ccRCC) initiation and
progression are being increasingly defined. The TRACERx Renal studies and others that …

VHL, the story of a tumour suppressor gene

L Gossage, T Eisen, ER Maher - Nature Reviews Cancer, 2015 - nature.com
Abstract Since the Von Hippel–Lindau (VHL) disease tumour suppressor gene VHL was
identified in 1993 as the genetic basis for a rare disorder, it has proved to be of wide medical …

The PI3K/AKT pathway and renal cell carcinoma

H Guo, P German, S Bai, S Barnes, W Guo, X Qi… - Journal of genetics and …, 2015 - Elsevier
The phosphatidylinositol 3 kinase (PI3K)/AKT pathway is genetically targeted in more
pathway components and in more tumor types than any other growth factor signaling …

New strategies in renal cell carcinoma: targeting the genetic and metabolic basis of disease

R Srinivasan, CJ Ricketts, C Sourbier… - Clinical cancer research, 2015 - AACR
The development of new forms of treatment of advanced renal cell carcinoma over the past
two decades has been primarily focused on targeting the VHL/HIF pathway. The recent …

Targeting tumor suppressor genes for cancer therapy

Y Liu, X Hu, C Han, L Wang, X Zhang, X He, X Lu - Bioessays, 2015 - Wiley Online Library
Cancer drugs are broadly classified into two categories: cytotoxic chemotherapies and
targeted therapies that specifically modulate the activity of one or more proteins involved in …

Deubiquitylase OTUD6B governs pVHL stability in an enzyme‐independent manner and suppresses hepatocellular carcinoma metastasis

X Liu, X Zhang, Z Peng, C Li, Z Wang… - Advanced …, 2020 - Wiley Online Library
Hypoxia inducible factors (HIFs) are the key transcription factors that allow cancer cells to
survive hypoxia. HIF's stability is mainly controlled by von Hippel–Lindau (pVHL)‐mediated …

Ubiquitination and regulation of AURKA identifies a hypoxia-independent E3 ligase activity of VHL

E Hasanov, G Chen, P Chowdhury, J Weldon, Z Ding… - Oncogene, 2017 - nature.com
The hypoxia-regulated tumor-suppressor von Hippel-Lindau (VHL) is an E3 ligase that
recognizes its substrates as part of an oxygen-dependent prolyl hydroxylase (PHD) reaction …

[HTML][HTML] USP9X destabilizes pVHL and promotes cell proliferation

C Zhang, Z Peng, M Zhu, P Wang, X Du, X Li, Y Liu… - Oncotarget, 2016 - ncbi.nlm.nih.gov
Numerous mutations of the Von Hippel-Lindau (VHL) gene have been reported to cause
dysfunction of VHL protein (pVHL) and lead to processes related to tumor progression …

Extraneuraxial hemangioblastoma: a clinicopathologic study of 10 cases with molecular analysis of the VHL gene

LA Muscarella, M Bisceglia, CA Galliani, N Zidar… - … -Research and Practice, 2018 - Elsevier
Less than 250 extraneuraxial hemangioblastomas occurring in paraneuraxial or peripheral
sites have been reported to date, sporadically or in the setting of von Hippel-Lindau disease …

The prefoldin complex stabilizes the von Hippel-Lindau protein against aggregation and degradation

F Chesnel, A Couturier, A Alusse, JP Gagné… - PLoS …, 2020 - journals.plos.org
Loss of von Hippel-Lindau protein pVHL function promotes VHL diseases, including
sporadic and inherited clear cell Renal Cell Carcinoma (ccRCC). Mechanisms controlling …