Deregulation of these two protein is involved in the development of VHL-associated clear-cell renal cell carcinoma (Maynard and Ohh, 2004) with pVHL ectopic expression in VHL−/− renal cell carcinoma leading to suppression of tumor formation in mice (Maynard and Ohh, 2004). The gene discussed is VHL; the disease is clear cell renal carcinoma.