[HTML][HTML] The VHL tumor suppressor gene: insights into oxygen sensing and cancer

WG Kaelin Jr - Transactions of the American Clinical and …, 2017 - ncbi.nlm.nih.gov
Mammalian cells sense changes in oxygen and transduce that information into adaptive
changes in gene expression using a conserved pathway that converges on the …

Targeting of HIF-α to the von Hippel-Lindau Ubiquitylation Complex by O2-Regulated Prolyl Hydroxylation

P Jaakkola, DR Mole, YM Tian, MI Wilson, J Gielbert… - Science, 2001 - science.org
Hypoxia-inducible factor (HIF) is a transcriptional complex that plays a central role in the
regulation of gene expression by oxygen. In oxygenated and iron replete cells, HIF-α …

Prolyl hydroxylase domain enzymes: important regulators of cancer metabolism

M Yang, H Su, T Soga, KR Kranc, PJ Pollard - Hypoxia, 2014 - Taylor & Francis
The hypoxia-inducible factor (HIF) prolyl hydroxylase domain enzymes (PHDs) regulate the
stability of HIF protein by post-translational hydroxylation of two conserved prolyl residues in …

Ubiquitination of hypoxia-inducible factor requires direct binding to the β-domain of the von Hippel–Lindau protein

M Ohh, CW Park, M Ivan, MA Hoffman, TY Kim… - Nature cell …, 2000 - nature.com
Abstract von Hippel–Lindau (VHL) disease is a hereditary cancer syndrome that is
characterized by the development of multiple vascular tumors and is caused by inactivation …

[HTML][HTML] Regulation of the hypoxia-inducible transcription factor 1α by the ubiquitin-proteasome pathway

PJ Kallio, WJ Wilson, S O'Brien, Y Makino… - Journal of Biological …, 1999 - ASBMB
HIF-1α (hypoxia-inducible factor 1α) is a basic-helix-loop-helix PAS (Per/Arnt/Sim)
transcription factor that, under hypoxic conditions, dimerizes with a partner factor, the basic …

[HTML][HTML] The von Hippel-Lindau protein interacts with heteronuclear ribonucleoprotein a2 and regulates its expression

PA Pioli, WFC Rigby - Journal of Biological Chemistry, 2001 - ASBMB
The product of the von Hippel-Lindau (VHL) tumor suppressor gene, pVHL, functions as a
ubiquitin-protein isopeptide ligase in regulating HIF-1 protein turnover, thus accounting for …

[HTML][HTML] Tumor suppression by the von Hippel-Lindau protein requires phosphorylation of the acidic domain

MP Lolkema, ML Gervais, CM Snijckers, RP Hill… - Journal of Biological …, 2005 - ASBMB
The tumor suppressor function of the von Hippel-Lindau protein (pVHL) has previously been
linked to its role in regulating hypoxia-inducible factor levels. However, VHL gene mutations …

Transgenic models to understand hypoxia‐inducible factor function

A Doedens, RS Johnson - Methods in enzymology, 2007 - Elsevier
The hypoxia‐inducible factor (HIF) is a heterodimeric basic helix‐loop‐helix (bHLH)
transcription factor that controls the mammalian cellular transcriptional response to low …

The LIMD1 protein bridges an association between the prolyl hydroxylases and VHL to repress HIF-1 activity

DE Foxler, KS Bridge, V James, TM Webb, M Mee… - Nature cell …, 2012 - nature.com
There are three prolyl hydroxylases (PHD1, 2 and 3) that regulate the hypoxia-inducible
factors (HIFs), the master transcriptional regulators that respond to changes in intracellular …

Accumulation of hypoxia-inducible factor-1α is limited by transcription-dependent depletion

ZN Demidenko, AM Rapisarda, M Garayoa… - Oncogene, 2005 - nature.com
In the presence of oxygen and iron, hypoxia-inducible factor (HIF-1α) is rapidly degraded via
the prolyl hydroxylases (PHD)/VHL pathways. Given striking similarities between p53 and …