ZFIN

ZFIN ID: ZDB-GENE-070112-1042

ZFIN ID: ZDB-GENE-070112-1042

CITATIONS (69 total)

Gene Name:	von Hippel-Lindau tumor suppressor
Gene Symbol:	vhl

Petzold, T., Brivio, S., Linnerz, T., Watanabe, M., Gerhardt, H., Bertrand, J.Y. (2025) Connexin 41.8 governs timely haematopoietic stem and progenitor cell specification. Biology Open. :

Wang, C., Rong, X., Zhang, H., Wang, B., Bai, Y., Sun, Y., Zhao, C., Zhou, J. (2025) pVHL regulates protein stability of the TCF/LEF transcription factor family via ubiquitin-independent proteasomal degradation. Cellular and molecular life sciences : CMLS. 82:335335

Yue, Y., Tang, Y., Huang, H., Zheng, D., Liu, C., Zhang, H., Liu, Y., Li, Y., Sun, X., Lu, L. (2023) VBP1 negatively regulates CHIP and selectively inhibits the activity of hypoxia-inducible factor (HIF)-1α but not HIF-2α. The Journal of biological chemistry. 299(6):104829

Zhang, R., Ma, Z., Wang, J., Fan, C. (2023) HIF signaling overactivation inhibits lateral line neuromast development through Wnt in zebrafish. Gene. 898:148077

Do, T.C., Lau, J.W., Sun, C., Liu, S., Kha, K.T., Lim, S.T., Oon, Y.Y., Kwan, Y.P., Ma, J.J., Mu, Y., Liu, X., Carney, T.J., Wang, X., Xing, B. (2022) Hypoxia deactivates epigenetic feedbacks via enzyme-derived clicking proteolysis-targeting chimeras. Science advances. 8:eabq2216

Samarasinghe, K.T.G., Jaime-Figueroa, S., Burgess, M., Nalawansha, D.A., Dai, K., Hu, Z., Bebenek, A., Holley, S.A., Crews, C.M. (2021) Targeted degradation of transcription factors by TRAFTACs: TRAnscription Factor TArgeting Chimeras. Cell chemical biology. 28(5):648-661.e5

Wrighton, P.J., Shwartz, A., Heo, J.M., Quenzer, E.D., LaBella, K.A., Harper, J.W., Goessling, W. (2021) Quantitative intravital imaging reveals in vivo dynamics of physiological-stress induced mitophagy. Journal of Cell Science. 134(4):

Wu, S., Chen, K., Xu, T., Ma, K., Gao, L., Fu, C., Zhang, W., Jing, C., Ren, C., Deng, M., Chen, Y., Zhou, Y., Pan, W., Jia, X. (2021) Tpr Deficiency Disrupts Erythroid Maturation With Impaired Chromatin Condensation in Zebrafish Embryogenesis. Frontiers in cell and developmental biology. 9:709923

Fan, S., Wang, J., Yu, G., Rong, F., Zhang, D., Xu, C., Du, J., Li, Z., Ouyang, G., Xiao, W. (2020) TET is targeted for proteasomal degradation by the PHD-pVHL pathway to reduce DNA hydroxymethylation. The Journal of biological chemistry. 295(48):16299-16313

Kim, H.R., Santhakumar, K., Markham, E., Baldera, D., Greenald, D., Bryant, H.E., El-Khamisy, S.F., van Eeden, F.J. (2020) Investigation of the role of VHL-HIF signaling in DNA repair and apoptosis in zebrafish. Oncotarget. 11:1109-1130

Klems, A., van Rijssel, J., Ramms, A.S., Wild, R., Hammer, J., Merkel, M., Derenbach, L., Préau, L., Hinkel, R., Suarez-Martinez, I., Schulte-Merker, S., Vidal, R., Sauer, S., Kivelä, R., Alitalo, K., Kupatt, C., van Buul, J.D., le Noble, F. (2020) The GEF Trio controls endothelial cell size and arterial remodeling downstream of Vegf signaling in both zebrafish and cell models. Nature communications. 11:5319

Lei, Z., Klasson, T.D., Brandt, M.M., van de Hoek, G., Logister, I., Cheng, C., Doevendans, P.A., Sluijter, J.P.G., Giles, R.H. (2020) Control of Angiogenesis via a VHL/miR-212/132 Axis. Cells. 9(4):

Marchi, D., Santhakumar, K., Markham, E., Li, N., Storbeck, K.H., Krone, N., Cunliffe, V.T., van Eeden, F.J.M. (2020) Bidirectional crosstalk between Hypoxia-Inducible Factor and glucocorticoid signalling in zebrafish larvae. PLoS Genetics. 16:e1008757

Ratnayake, R., Gunasekera, S.P., Ma, J.J., Dang, L.H., Carney, T., Paul, V.J., Luesch, H. (2020) Dolastatin 15 from a Marine Cyanobacterium Suppresses HIF-1α Mediated Cancer Cell Viability and Vascularization. Chembiochem : a European journal of chemical biology. 21(16):2356-2366

Sundaramurthi, H., Roche, S.L., Grice, G.L., Moran, A., Dillion, E.T., Campiani, G., Nathan, J.A., Kennedy, B.N. (2020) Selective Histone Deacetylase 6 Inhibitors Restore Cone Photoreceptor Vision or Outer Segment Morphology in Zebrafish and Mouse Models of Retinal Blindness. Frontiers in cell and developmental biology. 8:689

Tu, J., Liu, X., Jia, H., Reilly, J., Yu, S., Cai, C., Liu, F., Lv, Y., Huang, Y., Lu, Z., Han, S., Jiang, T., Shu, X., Wu, X., Tang, Z., Lu, Q., Liu, M. (2020) The chromatin remodeler Brg1 is required for formation and maintenance of hematopoietic stem cells. FASEB journal : official publication of the Federation of American Societies for Experimental Biology. 34(9):11997-12008

Ward, R., Ali, Z., Slater, K., Reynolds, A.L., Jensen, L.D., Kennedy, B.N. (2019) Pharmacological restoration of visual function in a zebrafish model of von-Hippel Lindau disease. Developmental Biology. 457(2):226-234

Gooskens, S.L., Klasson, T.D., Gremmels, H., Logister, I., Pieters, R., Perlman, E.J., Giles, R.H., van den Heuvel-Eibrink, M.M. (2018) TCF21 hypermethylation regulates renal tumor cell clonogenic proliferation and migration. Molecular Oncology. 12:166-179

Greenhough, A., Bagley, C., Heesom, K.J., Gurevich, D.B., Gay, D., Bond, M., Collard, T.J., Paraskeva, C., Martin, P., Sansom, O.J., Malik, K., Williams, A.C. (2018) Cancer cell adaptation to hypoxia involves a HIF-GPRC5A-YAP axis. EMBO Molecular Medicine. 10(11):

van Rooijen, E., van de Hoek, G., Logister, I., Ajzenberg, H., Knoers, N.V.A.M., van Eeden, F., Voest, E.E., Schulte-Merker, S., Giles, R.H. (2018) The von Hippel-Lindau Gene Is Required to Maintain Renal Proximal Tubule and Glomerulus Integrity in Zebrafish Larvae. Nephron. 138(4):310-323

Lim, S.E., Esain, V., Kwan, W., Theodore, L.N., Cortes, M., Frost, I.M., Liu, S.Y., North, T.E. (2017) HIF1α-induced PDGFRβ signaling promotes developmental HSC production via IL-6 activation. Experimental hematology. 46:83-95.e6

Vettori, A., Greenald, D., Wilson, G.K., Peron, M., Facchinello, N., Markham, E., Sinnakaruppan, M., Matthews, L.C., McKeating, J.A., Argenton, F., van Eeden, F.J.M. (2017) Glucocorticoids promote Von Hippel Lindau degradation and Hif-1α stabilization. Proceedings of the National Academy of Sciences of the United States of America. 114(37):9948-9953

Wild, R., Klems, A., Takamiya, M., Hayashi, Y., Strähle, U., Ando, K., Mochizuki, N., van Impel, A., Schulte-Merker, S., Krueger, J., Preau, L., le Noble, F. (2017) Neuronal sFlt1 and Vegfaa determine venous sprouting and spinal cord vascularization. Nature communications. 8:13991

Bousquet, M.S., Ma, J.J., Ratnayake, R., Havre, P.A., Yao, J., Dang, N.H., Paul, V.J., Carney, T.J., Dang, L.H., Luesch, H. (2016) Multidimensional Screening Platform for Simultaneously Targeting Oncogenic KRAS and Hypoxia-Inducible Factors Pathways in Colorectal Cancer. ACS Chemical Biology. 11(5):1322-31

Brunt, L.H., Skinner, R.E., Roddy, K.A., Araujo, N.M., Rayfield, E.J., Hammond, C.L. (2016) Differential effects of altered patterns of movement and strain on joint cell behaviour and skeletal morphogenesis. Osteoarthritis and Cartilage. 24(11):1940-1950

Bräutigam, L., Pudelko, L., Jemth, A.S., Gad, H., Narwal, M., Gustafsson, R., Karsten, S., Carreras-Puigvert, J., Homan, E., Berndt, C., Warpman Berglund, U., Stenmark, P., Helleday, T. (2016) Hypoxic signaling and the cellular redox tumor environment determine sensitivity to MTH1 inhibition. Cancer research. 76(8):2366-75

Jain, I.H., Zazzeron, L., Goli, R., Alexa, K., Schatzman-Bone, S., Dhillon, H., Goldberger, O., Peng, J., Shalem, O., Sanjana, N.E., Zhang, F., Goessling, W., Zapol, W.M., Mootha, V.K. (2016) Hypoxia as a therapy for mitochondrial disease. Science (New York, N.Y.). 352(6281):54-61

Liu, X., Cai, X., Hu, B., Mei, Z., Zhang, D., Ouyang, G., Wang, J., Zhang, W., Xiao, W. (2016) Forkhead transcription factor 3a (FOXO3a) modulates hypoxia signaling via up-regulation of von Hippel-Lindau gene (VHL). The Journal of biological chemistry. 291(49):25692-25705

Noonan, H.R., Metelo, A.M., Kamei, C.N., Peterson, R.T., Drummond, I.A., Iliopoulos, O. (2016) Loss of vhl in the zebrafish pronephros recapitulates early stages of human clear cell renal cell carcinoma. Disease models & mechanisms. 9:873-884

Chen, Y.H., Chang, C.F., Lai, Y.Y., Sun, C.Y., Ding, Y.J., Tsai, J.N. (2015) von Hippel-Lindau gene plays a role during zebrafish pronephros development. In vitro cellular & developmental biology. Animal. 51(10):1023-32

Du, J., Zhang, D., Zhang, W., Ouyang, G., Wang, J., Liu, X., Li, S., Ji, W., Liu, W., Xiao, W. (2015) pVHL Negatively Regulates Antiviral Signaling by Targeting MAVS for Proteasomal Degradation. Journal of immunology (Baltimore, Md. : 1950). 195(4):1782-90

Elkon, R., Milon, B., Morrison, L., Shah, M., Vijayakumar, S., Racherla, M., Leitch, C.C., Silipino, L., Hadi, S., Weiss-Gayet, M., Barras, E., Schmid, C.D., Ait-Lounis, A., Barnes, A., Song, Y., Eisenman, D.J., Eliyahu, E., Frolenkov, G.I., Strome, S.E., Durand, B., Zaghloul, N.A., Jones, S.M., Reith, W., Hertzano, R. (2015) RFX transcription factors are essential for hearing in mice. Nature communications. 6:8549

Essers, P.B., Klasson, T.D., Pereboom, T.C., Mans, D.A., Nicastro, M., Boldt, K., Giles, R.H., and MacInnes, A.W. (2015) The von Hippel-Lindau tumor suppressor regulates programmed cell death 5-mediated degradation of Mdm2. Oncogene. 146(3):789-800.e8

Greenald, D., Jeyakani, J., Pelster, B., Sealy, I., Mathavan, S., van Eeden, F.J. (2015) Genome-wide mapping of Hif-1α binding sites in zebrafish. BMC Genomics. 16:923

Hsu, C.H., Lin, J.S., Lai, K.P., Li, J.W., Chan, T.F., You, M.S., Tse, W.K., Jiang, Y.J. (2015) A new mib allele with a chromosomal deletion covering foxc1a exhibits anterior somite specification defect.. Scientific Reports. 5:10673

Jensen, L.D., Nakamura, M., Bräutigam, L., Li, X., Liu, Y., Samani, N.J., Cao, Y. (2015) VEGF-B-Neuropilin-1 signaling is spatiotemporally indispensable for vascular and neuronal development in zebrafish. Proceedings of the National Academy of Sciences of the United States of America. 112(44):E5944-53

Martins Metelo, A., Noonan, H.R., Xiang, L., Jin, Y., Baker, R., Kamentsky, L., Zhang, Y., van Rooijen, E., Shin, J., Carpenter, A.E., Yeh, J.R., Peterson, R.T., Iliopoulos, O. (2015) Pharmacological HIF2α inhibition improves VHL disease–associated phenotypes in zebrafish model. J. Clin. Invest.. 125(5):1987-97

Novodvorsky, P., Watson, O., Gray, C., Wilkinson, R.N., Reeve, S., Smythe, C., Beniston, R., Plant, K., Maguire, R., M K Rothman, A., Elworthy, S., van Eeden, F.J., Chico, T.J. (2015) klf2ash317 Mutant Zebrafish Do Not Recapitulate Morpholino-Induced Vascular and Haematopoietic Phenotypes. PLoS One. 10:e0141611

So, J.H., Kim, J.D., Yoo, K.W., Kim, H.T., Jung, S.H., Choi, J.H., Lee, M.S., Jin, S.W., Kim, C.H. (2014) FIH-1, a Novel Interactor of Mindbomb, Functions as an Essential Anti-Angiogenic Factor during Zebrafish Vascular Development. PLoS One. 9:e109517

Bonventre, J.A., Kung, T.S., White, L.A., and Cooper, K.R. (2013) Manipulation of the HIF-Vegf pathway rescues methyl tert-butyl ether (MTBE)-induced vascular lesions. Toxicology and applied pharmacology. 273(3):623-34

Harris, J.M., Esain, V., Frechette, G.M., Harris, L.J., Cox, A.G., Cortes, M., Garnaas, M.K., Carroll, K.J., Cutting, C.C., Khan, T., Elks, P.M., Renshaw, S.A., Dickinson, B.C., Chang, C.J., Murphy, M.P., Paw, B.H., Vander Heiden, M.G., Goessling, W., and North, T.E. (2013) Glucose metabolism impacts the spatio-temporal onset and magnitude of HSC induction in vivo. Blood. 121(13):2483-2493

Mans, D.A., Vermaat, J.S., Weijts, B.G., van Rooijen, E., van Reeuwijk, J., Boldt, K., Daenen, L.G., van der Groep, P., Rowland, B.D., Jans, J.J., Roepman, R., Voest, E.E., van Diest, P.J., Verhaar, M.C., de Bruin, A., and Giles, R.H. (2013) Regulation of E2F1 by the von Hippel-Lindau tumour suppressor protein predicts survival in renal cell cancer patients. The Journal of pathology. 231(1):117-29

Watson, O., Novodvorsky, P., Gray, C., Rothman, A.M., Lawrie, A., Gerhardt, H., Crossman, D.C., Haase, A., McMahon, K., Gering, M., van Eeden, F.J., and Chico, T.J. (2013) Blood flow suppresses vascular Notch signalling via dll4 and is required for angiogenesis in response to hypoxic signalling. Cardiovascular research. 100(2):252-61

Wilkinson, R.N., Elworthy, S., Ingham, P.W., and van Eeden, F.J. (2013) A method for high-throughput PCR-based genotyping of larval zebrafish tail biopsies. Biotechniques. 55(6):314-316

Santhakumar, K., Judson, E.C., Elks, P.M., McKee, S., Elworthy, S., van Rooijen, E., Walmsley, S.S., Renshaw, S., Cross, S.S., and van Eeden, F.J. (2012) A zebrafish model to study and therapeutically manipulate hypoxia signaling in tumorigenesis. Cancer research. 72(16):4017-4027

van der Velden, Y.U., Wang, L., Zevenhoven, J., van Rooijen, E., van Lohuizen, M., Giles, R.H., Clevers, H., and Haramis, A.P. (2011) The serine-threonine kinase LKB1 is essential for survival under energetic stress in zebrafish. Proceedings of the National Academy of Sciences of the United States of America. 108(11):4358-4363

van Rooijen, E., Voest, E.E., Logister, I., Bussmann, J., Korving, J., van Eeden, F.J., Giles, R.H., and Schulte-Merker, S. (2010) von Hippel-Lindau tumor suppressor mutants faithfully model pathological hypoxia-driven angiogenesis and vascular retinopathies in zebrafish. Disease models & mechanisms. 3(5-6):343-353

Yaqoob, N., and Schwerte, T. (2010) Cardiovascular and respiratory developmental plasticity under oxygen depleted environment and in genetically hypoxic zebrafish (Danio rerio). Comparative biochemistry and physiology. Part A, Molecular & integrative physiology. 156(4):475-484

van Rooijen, E., Voest, E.E., Logister, I., Korving, J., Schwerte, T., Schulte-Merker, S., Giles, R.H., and van Eeden, F.J. (2009) Zebrafish mutants in the von Hippel-Lindau (VHL) tumor suppressor display a hypoxic response and recapitulate key aspects of Chuvash polycythemia. Blood. 113(25):6449-6460

Strausberg,R.L., Feingold,E.A., Grouse,L.H., Derge,J.G., Klausner,R.D., Collins,F.S., Wagner,L., Shenmen,C.M., Schuler,G.D., Altschul,S.F., Zeeberg,B., Buetow,K.H., Schaefer,C.F., Bhat,N.K., Hopkins,R.F., Jordan,H., Moore,T., Max,S.I., Wang,J., Hsieh,F., Diatchenko,L., Marusina,K., Farmer,A.A., Rubin,G.M., Hong,L., Stapleton,M., Soares,M.B., Bonaldo,M.F., Casavant,T.L., Scheetz,T.E., Brownstein,M.J., Usdin,T.B., Toshiyuki,S., Carninci,P., Prange,C., Raha,S.S., Loquellano,N.A., Peters,G.J., Abramson,R.D., Mullahy,S.J., Bosak,S.A., McEwan,P.J., McKernan,K.J., Malek,J.A., Gunaratne,P.H., Richards,S., Worley,K.C., Hale,S., Garcia,A.M., Gay,L.J., Hulyk,S.W., Villalon,D.K., Muzny,D.M., Sodergren,E.J., Lu,X., Gibbs,R.A., Fahey,J., Helton,E., Ketteman,M., Madan,A., Rodrigues,S., Sanchez,A., Whiting,M., Madan,A., Young,A.C., Shevchenko,Y., Bouffard,G.G., Blakesley,R.W., Touchman,J.W., Green,E.D., Dickson,M.C., Rodriguez,A.C., Grimwood,J., Schmutz,J., Myers,R.M., Butterfield,Y.S., Krzywinski,M.I., Skalska,U., Smailus,D.E., Schnerch,A., Schein,J.E., Jones,S.J., and Marra,M.A. (2002) Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences. Proceedings of the National Academy of Sciences of the United States of America. 99(26):16899-903

Additional Citations (19):

ZFIN Staff (2023) Automated Curation of UniProt Database Links. Automated Data Submission.

ZFIN Staff (2022) Electronic Gene Ontology annotations created by ARBA machine learning models. Automated Data Submission.

ZFIN Staff (2020) Addition of links from ZFIN to Expression Atlas. Semi-automated Curation.

ZFIN Staff (2017) Curation of PANTHER Gene IDs. Automated Data Submission.

ZFIN Staff (2017) Curation of Alliance of Genome Resources Database Links. Automated Data Submission.

ZFIN Staff (2015) Data Model Change: Sequence Targeting Reagents Removed from Environment. ZFIN Historical Data.

Busch-Nentwich, E., Kettleborough, R., Dooley, C. M., Scahill, C., Sealy, I., White, R., Herd, C., Mehroke, S., Wali, N., Carruthers, S., Hall, A., Collins, J., Gibbons, R., Pusztai, Z., Clark, R., and Stemple, D.L. (2013) Sanger Institute Zebrafish Mutation Project mutant data submission. ZFIN Direct Data Submission.

ZFIN Staff (2013) Semi-automated association of ENSDARG identifiers with ZFIN genes for the ZMP project. Semi-automated Curation.

UniProt-GOA (2011) Gene Ontology annotation based on the automatic assignment of UniProtKB Subcellular Location terms in UniProtKB/TrEMBL entries. Manually curated data.

Busch-Nentwich, E., Kettleborough, R., Fenyes, F., Herd, C., Collins, J., de Bruijn, E., van Eeden, F., Cuppen, E., and Stemple, D.L. (2010) Sanger Institute Zebrafish Mutation Resource targeted knock-out mutants phenotype and image data submission, Sanger Institute Zebrafish Mutation Resource and Hubrecht laboratory. ZFIN Direct Data Submission.

Gaudet, P., Livstone, M., Thomas, P., The Reference Genome Project (2010) Annotation inferences using phylogenetic trees. Automated Data Submission.

Ensembl curators, GOA curators (2006) Automatic transfer of experimentally verified manual GO annotation data to orthologs using Ensembl Compara. Manually curated data.

ZFIN Staff (2006) Curation of Ensembl Database Links. Automated Data Submission.

ZFIN Staff (2004) ZGC Data Curation and Association in ZFIN by ZFIN Staff. Semi-automated Curation.

Zebrafish Nomenclature Committee (2003) Nomenclature Data Curation (2003-2010). Nomenclature Committee Submission.

ZFIN Staff (2003) Curation of VEGA Database Links. Automated Data Submission.

ZFIN Staff (2003) Curation of orthology data. Manually curated data.

ZFIN Staff (2002) Curation of NCBI Gene Data Via Shared RNA Sequence IDs. Automated Data Submission.

ZFIN Staff (2002) Scientific Curation. Manually curated data.