Nature Communications (Nov 2021)

BAF complexes drive proliferation and block myogenic differentiation in fusion-positive rhabdomyosarcoma

  • Dominik Laubscher,
  • Berkley E. Gryder,
  • Benjamin D. Sunkel,
  • Thorkell Andresson,
  • Marco Wachtel,
  • Sudipto Das,
  • Bernd Roschitzki,
  • Witold Wolski,
  • Xiaoli S. Wu,
  • Hsien-Chao Chou,
  • Young K. Song,
  • Chaoyu Wang,
  • Jun S. Wei,
  • Meng Wang,
  • Xinyu Wen,
  • Quy Ai Ngo,
  • Joana G. Marques,
  • Christopher R. Vakoc,
  • Beat W. Schäfer,
  • Benjamin Z. Stanton,
  • Javed Khan

DOI
https://doi.org/10.1038/s41467-021-27176-w
Journal volume & issue
Vol. 12, no. 1
pp. 1 – 16

Abstract

Read online

Rhabdomyosarcoma (RMS) is a pediatric malignancy of skeletal muscle lineage with an aggressive subtype caused by translocations involving PAX3- /PAX7-FOXO1 chimeric transcription factors. Here the authors show that the BRG1-containing BAF complex is overexpressed and acts largely independently of the PAX3-FOXO1 chimera on chromatin to result in a myogenic differentiation blockade in this malignancy.