*3.4. CRL2VHL*

The tumor suppressor protein von Hippel–Lindau (VHL) is a component of an E3 ubiquitin ligase complex that also contains the sca ffold protein Cullin 2 and RBX1 [151]. Mutations in VHL related to formation of the E3 ubiquitin ligase complex lead to von Hippel–Lindau syndrome, which can exhibit both ciliopathy and cancer phenotypes [140,152]. CRL2VHL ubiquitinates the α subunit of the transcription factor hypoxia-inducible factor 1 (HIF1 α), leading to its proteasomal degradation [138,153]. VHL and HIF1 α positively and negatively, respectively, regulate ciliogenesis and cancer [107,139,140,154].
