INI-1

Background

INI-1 gene is a tumor-suppressor gene located on chromosome 22q11.2. It is one of the members of SWI / SWF chromatin remodeling complex and its gene product is ubiquitously expressed in nuclei of all normal tissues. The INI-1 gene encodes an hSWI/SNF chromatin remodeling complex protein with unknown function, which is prone to mutation and deletion in malignant rhabdomyoid tumor (MRT). The gene is inactivated in most malignant rhabdomyomas (including atypical teratoid and rhabdoid tumor) and epithelioid sarcoma, so negative expression is helpful for diagnosis, while most medulloblastomas and primitive neuroectodermal tumors express INI-1.