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Gene Review

Vhl  -  von Hippel-Lindau tumor suppressor, E3...

Rattus norvegicus

Synonyms: Von Hippel-Lindau disease tumor suppressor, pVHL
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Disease relevance of Vhl

  • Thus, loss of pVHL function may be causative in pheochromocytoma-associated hypercatecholaminemia and arterial hypertension [1].
  • A decrease in pVHL accumulation also resulted in an increase in TH mRNA accumulation and transcription of the TH gene during hypoxia [1].
  • It has been shown that pVHL can suppress the expression of certain genes that are overexpressed in renal carcinomas [2].

High impact information on Vhl

  • PC12 cells that express VHL antisense RNA had 5-10-fold reduced levels of endogenous pVHL and 2-3-fold increased levels of TH protein and mRNA [1].
  • This is the first evidence that endogenous pVHL is a physiological regulator of the catecholaminergic phenotype [1].
  • In addition, neural progenitor cells expressing pVHL following gene transfer showed distinct neuronal differentiation and no induction of HIF-1alpha under the normoxic condition but not under the anoxic condition [3].
  • Transient transfection analysis utilizing PDGF-B promoter-chloramphenicol acetyl transferase (CAT) reporter constructs revealed that pVHL inhibition of PDGF-B expression is mediated via the Sp1-binding element in the proximal region of the PDGF-B promoter [2].
  • Further analysis of the HIF-alpha degradation regulation revealed that prolyl 4-hydroxylase (P4ha1) and von Hippel-Lindau syndrome homolog (Vhl) were both up-regulated at the troughs compared with the peaks [4].

Other interactions of Vhl

  • We also show that some of the effects of pVHL on activity of the TH promoter are mediated through HIFs [5].


  1. Endogenous von Hippel-Lindau tumor suppressor protein regulates catecholaminergic phenotype in PC12 cells. Bauer, A.L., Paulding, W.R., Striet, J.B., Schnell, P.O., Czyzyk-Krzeska, M.F. Cancer Res. (2002) [Pubmed]
  2. von Hippel-Lindau tumor suppressor protein represses platelet-derived growth factor B-chain gene expression via the Sp1 binding element in the proximal PDGF-B promoter. Rafty, L.A., Khachigian, L.M. J. Cell. Biochem. (2002) [Pubmed]
  3. The role of von Hippel-Lindau protein in the differentiation of neural progenitor cells under normoxic and anoxic conditions. Tanaka, Y., Kanno, H., Dezawa, M., Mimura, T., Kubo, A., Yamamoto, I. Neurosci. Lett. (2005) [Pubmed]
  4. Liver hypoxia and lack of recovery after reperfusion at high blood alcohol levels in the intragastric feeding model of alcohol liver disease. Li, J., French, B., Wu, Y., Vanketesh, R., Montgomery, R., Bardag-Gorce, F., Kitto, J., French, S.W. Exp. Mol. Pathol. (2004) [Pubmed]
  5. Regulation of tyrosine hydroxylase promoter activity by the von Hippel-Lindau tumor suppressor protein and hypoxia-inducible transcription factors. Schnell, P.O., Ignacak, M.L., Bauer, A.L., Striet, J.B., Paulding, W.R., Czyzyk-Krzeska, M.F. J. Neurochem. (2003) [Pubmed]
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