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 …

Inhibition of respiration extends C. elegans life span via reactive oxygen species that increase HIF-1 activity

SJ Lee, AB Hwang, C Kenyon - Current Biology, 2010 - cell.com
A mild inhibition of mitochondrial respiration extends the life span of many organisms,
including yeast, worms, flies, and mice [1–10], but the underlying mechanism is unknown …

Regulation of osteogenesis‐angiogenesis coupling by HIFs and VEGF

E Schipani, C Maes, G Carmeliet… - Journal of bone and …, 2009 - academic.oup.com
Bone is a highly vascularized tissue, but the function of angiogenesis in bone modeling and
remodeling is still poorly defined, and the molecular mechanisms that regulate angiogenesis …

Oxygen-dependent regulation of mitochondrial respiration by hypoxia-inducible factor 1

GL Semenza - Biochemical Journal, 2007 - portlandpress.com
The survival of metazoan organisms is dependent upon the utilization of O2 as a substrate
for COX (cytochrome c oxidase), which constitutes Complex IV of the mitochondrial …

Hypoxia-driven pathways in bone development, regeneration and disease

C Maes, G Carmeliet, E Schipani - Nature Reviews Rheumatology, 2012 - nature.com
Adaptation to hypoxia is a critical cellular event both in pathological settings, such as cancer
and ischaemia, and in normal development and differentiation. Oxygen is thought to be not …

Generating specificity and diversity in the transcriptional response to hypoxia

U Lendahl, KL Lee, H Yang, L Poellinger - Nature Reviews Genetics, 2009 - nature.com
The sensing of oxygen levels and maintenance of oxygen homeostasis is crucial for cells.
The hypoxic-sensitive regulation of gene expression allows information about the oxygen …

Genetic analysis of von Hippel‐Lindau disease

M Nordstrom‐O'Brien, RB van der Luijt… - Human …, 2010 - Wiley Online Library
Mutations in the von Hippel-Lindau (VHL) gene are responsible for VHL disease, congenital
polycythemia, and are found in many sporadic tumor types as well. Reports of VHL …

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 …