倒下，但未出局：SMARCB1 在滑膜肉瘤中的作用。

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

机构信息

Biological Sciences Graduate Program, University of California, San Diego, La Jolla, California.

Molecular and Cell Biology Laboratory, Salk Institute for Biological Studies, La Jolla, California.

出版信息

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

DOI:10.1158/2159-8290.CD-21-0591

PMID:34598951

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 , 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..

摘要

SMARCB1 是 BAF 复合物（也称为 SWI/SNF）的抑癌蛋白，其表达减少常见于滑膜肉瘤，这是一种由致瘤性 SS18-SSX 融合驱动的软组织恶性肿瘤，其与野生型 SS18 竞争 BAF 复合物的结合。在本期《细胞》杂志中，Li 和 Mulvihill 揭示了在表达 SS18-SSX2 融合蛋白的小鼠模型中，低表达的 SMARCB1 在滑膜肉瘤发生中有其功能作用，并提供了证据表明滑膜肉瘤中 SMARCB1 的减少是由于经典 BAF 复合物的整体降解。