Direct evidence for inherited predisposition to RCC is provided by a number of rare cancer syndromes with defined germline mutations in 11 genes (BAP1, FLCN, FH, MET, PTEN, SDHB, SDHC, SDHD, TSC1, TSC2 and VHL), that are associated with the development of different RCC subtypes4, 5. The gene discussed is BAP1; the disease is renal cell carcinoma.