Gene Symbol: vhl
Description: von Hippel-Lindau tumor suppressor
Alias: zgc:158722, von Hippel-Lindau disease tumor suppressor
Species: zebrafish

Top Publications

  1. van Rooijen E, Voest E, Logister I, Bussmann J, Korving J, van Eeden F, et al. von Hippel-Lindau tumor suppressor mutants faithfully model pathological hypoxia-driven angiogenesis and vascular retinopathies in zebrafish. Dis Model Mech. 2010;3:343-53 pubmed publisher
    Biallelic inactivation of the von Hippel-Lindau (VHL) tumor suppressor gene predisposes human patients to the development of highly vascularized neoplasms in multiple organ systems...
  2. Yaqoob N, Schwerte T. Cardiovascular and respiratory developmental plasticity under oxygen depleted environment and in genetically hypoxic zebrafish (Danio rerio). Comp Biochem Physiol A Mol Integr Physiol. 2010;156:475-84 pubmed publisher
    ..PO(2)=0 Torr, 0 kPa)-induced suspended animation at 4 dpf and iii) oxygen consumption in 8 dpf von Hippel-Lindau (vhl(-)(/)(-)) mutant that exhibits an artificial hypoxic response under normoxic conditions...
  3. van der Velden Y, Wang L, Zevenhoven J, van Rooijen E, Van Lohuizen M, Giles R, et al. The serine-threonine kinase LKB1 is essential for survival under energetic stress in zebrafish. Proc Natl Acad Sci U S A. 2011;108:4358-63 pubmed publisher
    ..rate in lkb1 mutants, either by application of the TOR inhibitor rapamycin or by crossing with von Hippel-Lindau (vhl) mutant fish (in which constitutive hypoxia signaling results in reduced metabolic rate), suppresses key aspects of ..
  4. Bonventre J, Kung T, White L, Cooper K. Manipulation of the HIF-Vegf pathway rescues methyl tert-butyl ether (MTBE)-induced vascular lesions. Toxicol Appl Pharmacol. 2013;273:623-34 pubmed publisher
    ..The selective toxicity of MTBE toward developing vasculature makes it a potentially useful chemical in the designing of new drugs or in elucidating roles for specific angiogenic proteins in future studies of vascular development. ..
  5. So J, Kim J, Yoo K, Kim H, Jung S, Choi J, et al. FIH-1, a novel interactor of mindbomb, functions as an essential anti-angiogenic factor during zebrafish vascular development. PLoS ONE. 2014;9:e109517 pubmed publisher
    ..Taken together, our data suggest that FIH-1 interacts with Mib E3 Ubiquitin ligase and modulates vascular development by attenuating VEGF-A signaling activity. ..
  6. Chen Y, Chang C, Lai Y, Sun C, Ding Y, Tsai J. von Hippel-Lindau gene plays a role during zebrafish pronephros development. In Vitro Cell Dev Biol Anim. 2015;51:1023-32 pubmed publisher
    ..In the present study, using zebrafish as a model, we showed that specific knockdown of endogenous vhl leads to pronephros malformation and renal failure...
  7. Wild R, Klems A, Takamiya M, Hayashi Y, Strahle U, Ando K, et al. Neuronal sFlt1 and Vegfaa determine venous sprouting and spinal cord vascularization. Nat Commun. 2017;8:13991 pubmed publisher
    ..Conceptually, our data suggest that spinal cord vascularization proceeds from veins involving two-tiered regulation of neuronal sFlt1 and Vegfaa via a novel sprouting mode. ..
  8. Novodvorsky P, Watson O, Gray C, Wilkinson R, Reeve S, Smythe C, et al. klf2ash317 Mutant Zebrafish Do Not Recapitulate Morpholino-Induced Vascular and Haematopoietic Phenotypes. PLoS ONE. 2015;10:e0141611 pubmed publisher
    ..klf2ash317 mutation did not reduce angiogenesis in zebrafish with homozygous mutations in von Hippel Lindau (vhl), a form of angiogenesis that is dependent on blood flow...
  9. Liu X, Cai X, Hu B, Mei Z, Zhang D, Ouyang G, et al. Forkhead Transcription Factor 3a (FOXO3a) Modulates Hypoxia Signaling via Up-regulation of the von Hippel-Lindau Gene (VHL). J Biol Chem. 2016;291:25692-25705 pubmed
    ..The von Hippel-Lindau (VHL) tumor suppressor gene encodes a component of the E3 ubiquitin ligase complex that mediates hypoxia-inducible ..

More Information


  1. Kim H, Greenald D, Vettori A, Markham E, Santhakumar K, Argenton F, et al. Zebrafish as a model for von Hippel Lindau and hypoxia-inducible factor signaling. Methods Cell Biol. 2017;138:497-523 pubmed publisher
    ..One component, von Hippel Lindau (VHL), is a well-known tumor suppressor, which acts at least in part via regulating HIF signaling...
  2. Santhakumar K, Judson E, Elks P, McKee S, Elworthy S, van Rooijen E, et al. A zebrafish model to study and therapeutically manipulate hypoxia signaling in tumorigenesis. Cancer Res. 2012;72:4017-27 pubmed publisher
    ..Zebrafish vhl mutants display a systemic hypoxia response, reflected by strong and ubiquitous transgene expression...
  3. Du J, Zhang D, Zhang W, Ouyang G, Wang J, Liu X, et al. pVHL Negatively Regulates Antiviral Signaling by Targeting MAVS for Proteasomal Degradation. J Immunol. 2015;195:1782-90 pubmed publisher
    The von Hippel-Lindau (VHL) gene is a well-defined tumor suppressor linked to human heredity cancer syndromes...
  4. Lim S, Esain V, Kwan W, Theodore L, Cortes M, Frost I, et al. HIF1?-induced PDGFR? signaling promotes developmental HSC production via IL-6 activation. Exp Hematol. 2017;46:83-95.e6 pubmed publisher
    ..Together, these findings define a Hif1?-regulated signaling axis mediated through PBFGB/PDGFR? and IL-6/IL-6R that acts to control embryonic HSPC production. ..
  5. Noonan H, Metelo A, Kamei C, Peterson R, Drummond I, Iliopoulos O. Loss of vhl in the zebrafish pronephros recapitulates early stages of human clear cell renal cell carcinoma. Dis Model Mech. 2016;9:873-84 pubmed publisher
    Patients with von Hippel-Lindau (VHL) disease harbor a germline mutation in the VHL gene leading to the development of several tumor types including clear cell renal cell carcinoma (ccRCC)...