The absence of RCC development by means of murine single Vhl disruption is presumed to be due to the fact that murine Vhl is located on chromosome (chr) 6, whereas Bap1 and Pbrm1 are located on chr 14, causing the avoidance of the simultaneous loss of Vhl and Bap1 or Pbrm1, while BAP1, PBRM1, and VHL are located on chr 3p21 and 3p25 and are frequently co-deleted in ccRCC [53]. Here, VHL is linked to nonpapillary renal cell carcinoma.