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In human cells, the best characterised function of pVHL is as a substrate recognition component of an E3 Ubiquitin ligase complex that also contains elongins B and C, Cul2 and Rbx1. The protein complex VBC targets proteinsfor ubiquitination-mediated degradation by the proteasome. We will investigate in human cells the ability of disease specific VHL mutations to support the assembly of the VBC complex and then to promote the ubiquitination and degradation of protein. We will express epitope-tagged mutated VHL in VHL-/- cells, purify the VHL-associated VBC complex and monitor its E3 ligase function using an in vitro HIF-1a ubiquitylation assay. Results will be confronted to individual patient clinical data