Recent exome sequencing studies led to the unexpected finding that SWI/SNF subunits are mutated at a high frequency in many different cancer types, and in particular, mutations in PBRM1, which encodes BAF180, were frequently identified, including in over 40% of renal cell carcinoma samples (Shain and Pollack, 2013, Varela et al., 2011, Xia et al., 2008), indicating that BAF180 plays a critical role in preventing tumorigenesis. This evidence concerns the gene PBRM1 and hereditary clear cell renal cell carcinoma.