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 von Hippel–Lindau tumour suppressor protein: O2 sensing and cancer

WG Kaelin Jr - Nature Reviews Cancer, 2008 - nature.com
Abstract The von Hippel–Lindau disease is caused by inactivating germline mutations of the
VHL tumour suppressor gene and is associated with an increased risk of a variety of …

VHL and HIF signalling in renal cell carcinogenesis

MM Baldewijns, IJH van Vlodrop… - The Journal of …, 2010 - Wiley Online Library
Hypoxia‐inducible factor (HIF) plays an important role in renal tumourigenesis. In the
majority of clear cell RCC (ccRCC), the most frequent and highly vascularized RCC subtype …

Von hippel-lindau disease

WG Kaelin Jr - Annu. Rev. Pathol. Mech. Dis., 2007 - annualreviews.org
Abstract von Hippel-Lindau disease, which is characterized by an increased risk of
hemangioblastomas, clear cell renal carcinomas, and pheochromocytomas, is caused by …

Von Hippel-Lindau disease: genetics and role of genetic counseling in a multiple neoplasia syndrome

SM Nielsen, L Rhodes, I Blanco, WK Chung… - Journal of Clinical …, 2016 - ascopubs.org
Von Hippel-Lindau disease (VHL) is one of the most common inherited neoplasia
syndromes and is characterized by highly vascular tumors of the eyes, brain, and spine, as …

Regulation of angiogenesis by hypoxia and hypoxia‐inducible factors

MM Hickey, MC Simon - Current topics in developmental biology, 2006 - Elsevier
Maintenance of oxygen homeostasis is critical for the survival of multicellular organs. As a
result, both invertebrates and vertebrates have developed highly specialized mechanisms to …

The VHL tumor suppressor and HIF: insights from genetic studies in mice

PP Kapitsinou, VH Haase - Cell Death & Differentiation, 2008 - nature.com
Abstract The von Hippel–Lindau tumor suppressor gene product, pVHL, functions as the
substrate recognition component of an E3-ubiquitin ligase, which targets the oxygen …

The von Hippel–Lindau Chuvash mutation promotes pulmonary hypertension and fibrosis in mice

MM Hickey, T Richardson, T Wang… - The Journal of …, 2010 - Am Soc Clin Investig
Mutation of the von Hippel–Lindau (VHL) tumor suppressor protein at codon 200 (R200W) is
associated with a disease known as Chuvash polycythemia. In addition to polycythemia …

Hypoxia-inducible factor underlies von Hippel-Lindau disease stigmata

M Ohh, CC Taber, FG Ferens, D Tarade - Elife, 2022 - elifesciences.org
von Hippel-Lindau (VHL) disease is a rare hereditary cancer syndrome that causes a
predisposition to renal clear-cell carcinoma, hemangioblastoma, pheochromocytoma, and …