vhl 1


Gene Symbol: vhl 1
Description: von Hippel-Lindau tumor suppressor homolog
Alias: von Hippel-Lindau tumor suppressor homolog
Species: Caenorhabditis elegans

Top Publications

  1. Epstein A, Gleadle J, McNeill L, Hewitson K, O ROURKE J, Mole D, et al. C. elegans EGL-9 and mammalian homologs define a family of dioxygenases that regulate HIF by prolyl hydroxylation. Cell. 2001;107:43-54 pubmed
  2. Shen C, Shao Z, Powell Coffman J. The Caenorhabditis elegans rhy-1 gene inhibits HIF-1 hypoxia-inducible factor activity in a negative feedback loop that does not include vhl-1. Genetics. 2006;174:1205-14 pubmed
    ..Collectively, these data support models in which RHY-1 and EGL-9 function in VHL-1-independent pathway(s) to repress HIF-1 transcriptional activity. ..
  3. Budde M, Roth M. Hydrogen sulfide increases hypoxia-inducible factor-1 activity independently of von Hippel-Lindau tumor suppressor-1 in C. elegans. Mol Biol Cell. 2010;21:212-7 pubmed publisher
    ..Because H(2)S is naturally produced by animal cells, our results suggest that endogenous H(2)S may influence HIF-1 activity. ..
  4. Ma D, Vozdek R, Bhatla N, Horvitz H. CYSL-1 interacts with the O2-sensing hydroxylase EGL-9 to promote H2S-modulated hypoxia-induced behavioral plasticity in C. elegans. Neuron. 2012;73:925-40 pubmed publisher
    ..These findings demonstrate that CYSL-1 acts to transduce signals from H(2)S to EGL-9 to regulate O(2)-dependent behavioral plasticity in C. elegans. ..
  5. Gharbi H, Fabretti F, Bharill P, Rinschen M, Brinkkötter S, Frommolt P, et al. Loss of the Birt-Hogg-Dubé gene product folliculin induces longevity in a hypoxia-inducible factor-dependent manner. Aging Cell. 2013;12:593-603 pubmed publisher
    ..These findings define FLCN as a player in HIF-dependent longevity signaling and connect organismal aging, stress resistance, and regulation of longevity with the formation of renal cell carcinoma. ..
  6. Bishop T, Lau K, Epstein A, Kim S, Jiang M, O Rourke D, et al. Genetic analysis of pathways regulated by the von Hippel-Lindau tumor suppressor in Caenorhabditis elegans. PLoS Biol. 2004;2:e289 pubmed
  7. Vasudevan S, Starostina N, Kipreos E. The Caenorhabditis elegans cell-cycle regulator ZYG-11 defines a conserved family of CUL-2 complex components. EMBO Rep. 2007;8:279-86 pubmed
    ..We show that the ZYG11 gene family encompasses two main branches in metazoa, and provide evidence that members of the extended ZYG11 family in nematodes and humans are conserved components of CUL2-based ubiquitin-ligases. ..
  8. Starostina N, Lim J, Schvarzstein M, Wells L, Spence A, Kipreos E. A CUL-2 ubiquitin ligase containing three FEM proteins degrades TRA-1 to regulate C. elegans sex determination. Dev Cell. 2007;13:127-39 pubmed
    ..This work demonstrates that the terminal step in C. elegans sex determination is controlled by ubiquitin-mediated proteolysis. ..
  9. Sendoel A, Kohler I, Fellmann C, Lowe S, Hengartner M. HIF-1 antagonizes p53-mediated apoptosis through a secreted neuronal tyrosinase. Nature. 2010;465:577-83 pubmed publisher
    ..Our findings identify a novel link between hypoxia and programmed cell death, and provide a paradigm for HIF-1 dictating apoptotic cell fate at a distance. ..

More Information


  1. Pocock R, Hobert O. Oxygen levels affect axon guidance and neuronal migration in Caenorhabditis elegans. Nat Neurosci. 2008;11:894-900 pubmed publisher
    ..Similar genetic pathways may cause aberrant human brain development under hypoxic conditions. ..
  2. Mehta R, Steinkraus K, Sutphin G, Ramos F, Shamieh L, Huh A, et al. Proteasomal regulation of the hypoxic response modulates aging in C. elegans. Science. 2009;324:1196-8 pubmed publisher
    ..VHL-1 and HIF-1 control longevity by a mechanism distinct from both dietary restriction and insulin-like signaling. These findings define VHL-1 and the hypoxic response as an alternative longevity and protein homeostasis pathway. ..