Down, but not out: A role for smarcb1 in synovial sarcoma

Abstract

Reduced protein expression of the BAF complex (also known as SWI/SNF) tumor suppressor SMARCB1 is frequently observed in human synovial sarcoma, a soft-tissue malignancy driven by the oncogenic SS18–SSX fusion, which competes with wild-type SS18 for BAF complex incorporation. In this issue of Cancer Discovery, Li and Mulvihill reveal that low-expressed SMARCB1 has a functional role in synovial sarcomagenesis in mouse models expressing the SS18–SSX2 fusion and present evidence that SMARCB1 reduction in synovial sarcoma is due to wholesale degradation of canonical BAF complexes.