SMARCB1 was the first BAF subunit identified as mutated in cancer and functions as a potent tumor suppressor: Smarcb1-null mice die early in embryogenesis (embryonic day 3.5–5), whereas Smarcb1-heterozygous mice rapidly develop aggressive tumors, including rhabdoid-like neoplasms (median onset ~11 weeks) [32]. This evidence concerns the gene SMARCB1 and neoplasm.