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Von Hippel-Lindau tumor suppressor

VHL, pVHL, von Hippel-Lindau tumor suppressor
Von Hippel-Lindau syndrome (VHL) is a dominantly inherited familial cancer syndrome predisposing to a variety of malignant and benign tumors. A germline mutation of this gene is the basis of familial inheritance of VHL syndrome. The protein encoded by this gene is a component of the protein complex that includes elongin B, elongin C, and cullin-2, and possesses ubiquitin ligase E3 activity. This protein is involved in the ubiquitination and degradation of hypoxia-inducible-factor (HIF), which is a transcription factor that plays a central role in the regulation of gene expression by oxygen. RNA polymerase II subunit POLR2G/RPB7 is also reported to be a target of this protein. Alternatively spliced transcript variants encoding distinct isoforms have been observed. [provided by RefSeq, Jul 2008] (from NCBI)
Top mentioned proteins: CAN, XRCC1, HAD, vascular endothelial growth factor, V1a
Papers using VHL antibodies
TS-1 enhances the effect of radiotherapy by suppressing radiation-induced hypoxia-inducible factor-1 activation and inducing endothelial cell apoptosis
Hiraoka M et al., In British Journal of Cancer, 2007
... cell carcinoma cell lines (RCC4) stably transfected with pcDNA3 (an empty vector) and pcDNA3-VHL (a VHL-expressing vector), respectively, were purchased from DS Pharma Biomedical (Osaka, Japan) ...
VHL loss actuates a HIF-independent senescence programme mediated by Rb and p400
Gnarra James R. et al., In Oncogene, 2007
... Anti-VHL (Ig32) was purchased from BD Pharmingen, anti-HA (Y-11), anti-cyclin B1, anti-cdc27 and anti-VHL (FL-181) antibodies were purchased from Santa Cruz Biotechnology, and anti-myc (9E10) antibody ...
The expression of the von Hippel-Lindau gene product and its impact on invasiveness of human breast cancer cells
Rozakis-Adcock M et al., In Oncogene, 2006
... (Santa Cruz, CA, USA); Phospho-Erk1/2 and Erk1/2 antibodies from Zymed Laboratories (San Francisco, CA, USA); VHL antibodies from BD Biosciences (San Jose, CA, USA) ...
C-terminal modifications regulate MDM2 dissociation and nuclear export of p53.
Jin Dong-Yan, In PLoS ONE, 2006
... The VHL antibodies were from Cell signaling technology Inc ...
TRAF6-mediated ubiquitination regulates nuclear translocation of NRIF, the p75 receptor interactor.
Koritzinsky Marianne, In PLoS ONE, 2004
... pVHL antibody was from BD Biosciences (Bedford, MA) ...
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Papers on VHL
Axitinib for the treatment of metastatic renal cell carcinoma.
Rini et al., Cleveland, United States. In Future Oncol, Feb 2016
UNASSIGNED: Renal cell carcinoma is a cancer that results from a genetic inactivation of the VHL tumor suppressor gene leading to an upregulation of VEGF.
An ID2-dependent mechanism for VHL inactivation in cancer.
Lasorella et al., New York City, United States. In Nature, Feb 2016
ID2 binds to the VHL ubiquitin ligase complex, displaces VHL-associated Cullin 2, and impairs HIF2α ubiquitylation and degradation.
Hypoxia Promotes Cancer Stem Cells via ID2-Dependent VHL Inactivation.
In Cancer Discov, Feb 2016
UNASSIGNED: Hypoxia inactivates DYRK1, preventing ID2 phosphorylation and promoting CSCs and HIF2α stabilization.
Formation of renal cysts and tumors in Vhl/Trp53-deficient mice requires HIF-1α and HIF-2α.
Frew et al., Zürich, Switzerland. In Cancer Res, Feb 2016
UNASSIGNED: The von Hippel-Lindau (VHL) tumor suppressor gene is inactivated in the majority of clear cell renal cell carcinomas (ccRCC), but genetic ablation of Vhl alone in mouse models is insufficient to recapitulate human tumorigenesis.
Von Hippel-Lindau disease: an evaluation of natural history and functional disability.
Opocher et al., Modena, Italy. In Neuro Oncol, Feb 2016
BACKGROUND: Although many studies have been published about specific lesions characterizing von Hippel-Lindau(VHL) disease, none have dealt with the natural history of the whole disease and the consequent disabilities.
Identification of DNA methylation-independent epigenetic events underlying clear cell renal cell carcinoma.
Liang et al., United States. In Cancer Res, Feb 2016
Compared to commonly mutated genes in ccRCC, such as the von Hippel-Lindau (VHL) tumor suppressor, the genes identified by AcceSssIble comprised distinct pathways and more frequently underwent epigenetic changes, suggesting that genetic and epigenetic alterations could be independent events in ccRCC.
Down-regulation of C12orf59 is associated with a poor prognosis and VHL mutations in renal cell carcinoma.
Li et al., Shenzhen, China. In Oncotarget, Feb 2016
VHL non-sense mutations or frame-shift mutations (P < 0.01), and UMPP gene non-sense mutations or frame-shift mutations (P = 0.01).
Jade-1: its structure, regulation and functions in the renal cancer.
Pei et al., Xuzhou, China. In Curr Mol Med, Dec 2015
Jade-1 is originally identified by the yeast two-hybrid system as a protein partner of von Hippel-Lindau (pVHL) tumor suppressor, a well-known renal tumor suppressor.
E3 Ubiquitin Ligase VHL Regulates Hypoxia-Inducible Factor-1α to Maintain Regulatory T Cell Stability and Suppressive Capacity.
Liu et al., Los Angeles, United States. In Immunity, Jul 2015
Here, we report that the E3 ubiquitin ligase VHL is essential for Treg cell function.
A lactate-induced response to hypoxia.
Yeom et al., Taejŏn, South Korea. In Cell, May 2015
We find that the NDRG3 protein is degraded in a PHD2/VHL-dependent manner in normoxia but is protected from destruction by binding to lactate that accumulates under hypoxia.
VHL, the story of a tumour suppressor gene.
Maher et al., Cambridge, United Kingdom. In Nat Rev Cancer, 2015
VHL tumour suppressor protein (pVHL) plays a key part in cellular oxygen sensing by targeting hypoxia-inducible factors for ubiquitylation and proteasomal degradation.
[Hereditary pheochromocytoma-associated syndromes. Part 1].
Beltsevich et al., In Ter Arkh, 2014
There have been the most investigated NF, RET, VHL, SDHD, SDHC, and SDHB gene mutations.
Von Hippel-Lindau disease.
Lonser et al., Bethesda, United States. In Handb Clin Neurol, 2014
von Hippel-Lindau (VHL) disease is an inheritable condition with an incidence of 1 in 36000 live births.
Molecular and immunologic markers of kidney cancer-potential applications in predictive, preventive and personalized medicine.
Gratchev et al., Mannheim, Germany. In Epma J, 2014
The most common molecular markers of kidney cancer include mutations and epigenetic inactivation of von Hippel-Lindau (VHL) gene, genes of vascular endothelial growth factor (VEGF) pathway, and carbonic anhydrase IX (CIAX).
TRPM3 and miR-204 establish a regulatory circuit that controls oncogenic autophagy in clear cell renal cell carcinoma.
Czyzyk-Krzeska et al., Cincinnati, United States. In Cancer Cell, 2014
The von Hippel-Lindau tumor suppressor (VHL) represses TRPM3 directly through miR-204 and indirectly through another miR-204 target, Caveolin 1 (CAV1).
Von Hippel-Lindau protein in the RPE is essential for normal ocular growth and vascular development.
Bainbridge et al., London, United Kingdom. In Development, 2012
The data showed that Von Hippel-Lindau protein-dependent regulation of Hif1a in the retinal pigment epithelium is essential for normal retinal pigment epithelial and iris development, ocular growth and vascular development in the anterior chamber.
Systemic VHL gene functions and the VHL disease.
Hsu et al., Boston, United States. In Febs Lett, 2012
functions of VHL likely play important roles in the development of VHL disease
Identification and functional characterization of pVHL-dependent cell surface proteins in renal cell carcinoma.
Moch et al., Zürich, Switzerland. In Neoplasia, 2012
pVHL-dependent cell surface glycoproteins as potential diagnostic markers for therapeutic targeting and RCC patient monitoring.
Knockdown of von Hippel-Lindau protein decreases lung cancer cell proliferation and colonization.
Zhou et al., Chicago, United States. In Febs Lett, 2012
Knockdown of pVHL decreased phosphorylation of FAK and expression of integrin, suggesting that pVHL regulates lung cancer development via integrin/FAK signaling pathway
[Localization of point mutations in the coding part of the VHL gene in clear cell renal cancer].
Zaletaev et al., In Mol Biol (mosk), 2012
39.1% samples with stage I harbor somatic mutations in VHL gene, however, no association with progression or metastases was found
More papers using VHL antibodies
Activation of the hypoxia-inducible factor-pathway and stimulation of angiogenesis by application of prolyl hydroxylase inhibitors.
Kalinichenko Vladimir V., In PLoS ONE, 2002
... Conditional VHL knockout mice were generated by crossbreeding three different transgenic mouse lines: a) floxed ...
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