Additionally, classic and more severe CSS are known to be caused by mutations in SMARCA4 (OMIM# 603254), the common core subunit SMARCB1 (OMIM# 601607), and accessory subunits such as SMARCE1 (OMIM# 603111), ARID1A (OMIM# 603024) and ARID2 (OMIM# 609539) [285]. This evidence concerns the gene SMARCE1 and Coffin-Siris syndrome.