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Gene Symbol SMARCB1
Synonyms BAF47 | CSS3 | hSNFS | INI-1 | INI1 | MRD15 | PPP1R144 | RDT | RTPS1 | Sfh1p | SNF5 | SNF5L1 | Snr1 | SWNTS1
Gene Description SMARCB1, SWI/SNF related, matrix associated, actin dependent regulator of chromatin, subfamily b, member 1, is a member of the SWI/SNF chromatin remodeling complex and regulates transcription of several genes involved in cell proliferation (PMID: 23095836, PMID: 28109176). Smarcb1 inactivation through loss is characteristic of atypical teratoid/rhabdoid tumors (PMID: 23095836), schwannomas (PMID: 28368924, PMID: 28824165), sinonasal carcinomas (PMID: 29797680), and colon carcinoma (PMID: 32083567).

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Clinical Trial Phase Therapies Title Recruitment Status Covered Countries Other Countries
NCT05286801 Phase Ib/II Atezolizumab + Tiragolumab Tiragolumab and Atezolizumab for the Treatment of Relapsed or Refractory SMARCB1 or SMARCA4 Deficient Tumors Recruiting USA | CAN | AUS 0
NCT05355753 Phase Ib/II CFT8634 A Study to Assess the Safety and Tolerability of CFT8634 in Locally Advanced or Metastatic SMARCB1-Perturbed Cancers, Including Synovial Sarcoma and SMARCB1-Null Tumors Terminated USA 0
NCT02601937 Phase I Tazemetostat A Phase 1 Study of the EZH2 Inhibitor Tazemetostat in Pediatric Subjects With Relapsed or Refractory INI1-Negative Tumors or Synovial Sarcoma Completed USA | NLD | ITA | GBR | FRA | DEU | CAN | AUS 1
NCT04416568 Phase II Ipilimumab + Nivolumab Study of Nivolumab and Ipilimumab in Children and Young Adults With INI1-Negative Cancers Recruiting USA 0
NCT04204941 Phase III Doxorubicin Doxorubicin + Tazemetostat Tazemetostat in Combination With Doxorubicin as Frontline Therapy for Advanced Epithelioid Sarcoma Recruiting USA | GBR | CAN 1
NCT02114229 Phase II Cisplatin + Cyclophosphamide + Methotrexate + Vincristine Sulfate Alisertib Alisertib + Carboplatin + Cyclophosphamide + Vincristine Sulfate Cyclophosphamide + Topotecan Alisertib + Carboplatin + Cyclophosphamide + Etoposide Carboplatin + Cyclophosphamide + Methotrexate + Vincristine Sulfate Alisertib + Cisplatin + Cyclophosphamide + Vincristine Sulfate Phase 2 Study of Alisertib Therapy for Rhabdoid Tumors (SJATRT) Active, not recruiting USA 0
NCT05715281 Phase II Atezolizumab + Tiragolumab Testing the Combination of Anti-cancer Drugs Atezolizumab and Tiragolumab in People With Advanced Stage Rare Cancers, RARE3 Trial Recruiting USA 0
NCT05407441 Phase Ib/II Ipilimumab + Nivolumab + Tazemetostat Tazemetostat+Nivo/Ipi in INI1-Neg/SMARCA4-Def Tumors Recruiting USA 0
NCT02601950 Phase II Tazemetostat A Study of Tazemetostat in Adult Participants With Soft Tissue Sarcoma Completed USA | ITA | GBR | FRA | DEU | CAN | BEL | AUS 1
NCT06444880 Phase II Cemiplimab + REGN4018 REGN4018 Phase II Trial of Ubamatamab Alone or in Combination With Cemiplimab in MUC16-Expressing SMARCB1-Deficient Malignancies Recruiting USA 0
NCT04965753 Phase I FHD-609 FHD-609 in Subjects With Advanced Synovial Sarcoma or Advanced SMARCB1-Loss Tumors Terminated USA | ITA | FRA | ESP 0
NCT03274258 Phase II Ipilimumab + Nivolumab Phase II Trial of Nivolumab Plus Ipilimumab in Patients With Renal Medullary Carcinoma Active, not recruiting USA 0
NCT03654716 Phase I ALRN-6924 + Cytarabine ALRN-6924 Phase 1 Study of the Dual MDM2/MDMX Inhibitor ALRN-6924 in Pediatric Cancer Completed USA 0
NCT03155620 Phase II Tazemetostat Larotrectinib LY3023414 Vemurafenib Palbociclib Olaparib Ulixertinib Erdafitinib Selumetinib Ensartinib Targeted Therapy Directed by Genetic Testing in Treating Pediatric Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphomas, or Histiocytic Disorders (The Pediatric MATCH Screening Trial) Recruiting USA | CAN | AUS 1
NCT03375307 Phase II Olaparib Testing Olaparib in Patients With Advanced or Metastatic (Cancer That Has Spread) Bladder Cancer and Other Genitourinary Tumors With DNA-Repair Genetic Changes Recruiting USA 0
NCT03587662 Phase II Gemcitabine + Ixazomib Doxorubicin + Gemcitabine + Ixazomib Ixazomib, Gemcitabine, and Doxorubicin in Treating Patients With Locally Advanced or Metastatic Kidney Cancer Active, not recruiting USA 0
NCT05347212 Phase II Nivolumab + Relatlimab Phase II Trial of Immunotherapy in Patients With Carcinomas Arising From the Renal Medulla Active, not recruiting USA 0
NCT03533582 Phase II Cisplatin + Doxorubicin + Gemcitabine + Oxaliplatin + Sorafenib Cisplatin + Doxorubicin + Fluorouracil + Vincristine Sulfate Carboplatin + Cisplatin + Doxorubicin + Etoposide Carboplatin + Cisplatin + Doxorubicin + Irinotecan + Vincristine Sulfate Cisplatin Cisplatin + Doxorubicin + Sorafenib Cisplatin + Doxorubicin Carboplatin + Cisplatin + Doxorubicin Cisplatin and Combination Chemotherapy in Treating Children and Young Adults With Hepatoblastoma or Liver Cancer After Surgery Active, not recruiting USA | NZL | CAN | AUS 2
NCT04266912 Phase Ib/II Avelumab + Berzosertib Avelumab and M6620 for the Treatment of DDR Deficient Metastatic or Unresectable Solid Tumors Active, not recruiting USA 0
NCT03213665 Phase II Tazemetostat Tazemetostat in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With EZH2, SMARCB1, or SMARCA4 Gene Mutations (A Pediatric MATCH Treatment Trial) Active, not recruiting USA 1