SMARCC2
Overview
SMARCC2 (SWI/SNF Related, Matrix Associated, Actin Dependent Regulator of Chromatin, Subfamily C, Member 2), also known as BAF170, encodes a structural subunit of the SWI/SNF chromatin-remodeling complex. As a fusion partner, SMARCC2 can create oncogenic kinase fusions when joined to receptor tyrosine kinases such as PDGFRB, generating constitutively active signaling.
Alterations observed in the corpus
- SMARCC2-PDGFRB fusion identified in a pediatric patient with B-cell lymphoblastic leukemia (BLL) in the PIPseq pediatric precision sequencing program (n=101, Columbia University); designated as a tyrosine kinase inhibitor (TKI) target. PMID:28007021
Cancer types (linked)
- BLL: SMARCC2-PDGFRB fusion creates a constitutively active PDGFRB kinase; TKI therapy (e.g., imatinib or dasatinib) is indicated. PMID:28007021
Co-occurrence and mutual exclusivity
- No additional co-mutation data reported in the corpus for this fusion case.
Therapeutic relevance
- SMARCC2-PDGFRB fusion is a targetable alteration; TKI inhibitors of PDGFRB (imatinib, dasatinib, ponatinib) are the relevant class; designated as a TKI target in the PIPseq cohort. PMID:28007021
Open questions
- Optimal TKI choice and efficacy in pediatric BLL with SMARCC2-PDGFRB fusion require prospective evaluation. PMID:28007021
Sources
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