Fig. 6: Proposed model for RSUME increase in VHL tumors and its action on VHL mutants function.
From: von Hippel-Lindau mutants in renal cell carcinoma are regulated by increased expression of RSUME
VHL missense Type 2 mutants fail in negatively regulate RSUME levels, leading to a permissive context for increased RSUME in VHL-related tumors, like RCC. By protein–protein interaction with VHL mutants, RSUME reduces HIF-2α-VHL binding and negatively regulates the assembly of the ECV complex. By this mean, RSUME regulates VHL Type 2 mutants loss of function and worsens HIF transcriptional activity deregulation, thus leading to increased angiogenesis and tumor progression
