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smarcb1
SMARCB1
SMARCB1 (INI1/BAF47) is a core SWI/SNF BAF complex subunit; biallelic LOF in ~100% AT/RT, rhabdoid tumors, and epithelioid sarcoma; SMARCB1 loss → PRC2/EZH2-mediated H3K27me3 at tumor suppressor loci; tazemetostat (EZH2 inhibitor) FDA-approved for epithelioid sarcoma.
Entry Metadata
| Field | Value |
|---|---|
| ID | smarcb1 |
| Name | SMARCB1 |
| Status | draft |
| Last reviewed | 2026-06-06 |
| Atlas | 01-human |
| Scale | 03-molecular |
Cross-Atlas Connections
Sources
- Versteege I, Sévenet N, Lange J, et al. Truncating mutations of hSNF5/INI1 in aggressive paediatric cancer. Nature. 1998;394(6689):203-206. · PubMed 9671307
- Gounder M, Schöffski P, Jones RL, et al. Tazemetostat in advanced epithelioid sarcoma with loss of INI1/SMARCB1: an international, open-label, phase 2 basket study. Lancet Oncol. 2020;21(11):1423-1432. · PubMed 33007258