Down, but Not Out: A Role for SMARCB1 in Synovial Sarcoma

Cancer Discov. 2021 Oct;11(10):2375-2377. doi: 10.1158/2159-8290.CD-21-0591.

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.See related article by Li et al., p. 2620.

Publication types

  • Comment

MeSH terms

  • Animals
  • Mice
  • Oncogene Proteins, Fusion / genetics
  • Sarcoma, Synovial* / genetics

Substances

  • Oncogene Proteins, Fusion