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Updated: Nov 30, 2025

Tumorsphere Derivation and Treatment from Primary Tumor Cells Isolated from Mouse Rhabdomyosarcomas
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Tumorsphere Derivation and Treatment from Primary Tumor Cells Isolated from Mouse Rhabdomyosarcomas

Published on: September 13, 2019

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HDAC6 promotes growth, migration/invasion, and self-renewal of rhabdomyosarcoma

Thao Q Pham1, Kristin Robinson1, Lin Xu2,3

  • 1Department of Laboratory Medicine and Pathology, University of Washington, Seattle, WA, USA.

Oncogene
|November 17, 2020
PubMed

Insights

Histone deacetylase 6 (HDAC6) drives pediatric rhabdomyosarcoma growth and spread. Targeting the HDAC6-RAC1 pathway offers a promising new therapy for this aggressive childhood cancer.

Area of Science:

  • Oncology
  • Molecular Biology
  • Pediatric Cancer Research

Background:

  • Rhabdomyosarcoma (RMS) is a severe pediatric cancer with poor outcomes for relapsed or metastatic cases.
  • Limited understanding of RMS progression hinders the development of effective targeted therapies.

Purpose of the Study:

  • To investigate the role of histone deacetylase 6 (HDAC6) in RMS progression.
  • To identify molecular targets for novel RMS therapies.

Main Methods:

  • In vitro and in vivo functional assays (zebrafish and xenograft mouse models).
  • Assessment of HDAC6-selective inhibitors.
  • Analysis of RAC1 GTPase function and expression.
  • Correlation of RAC1 expression with clinical prognosis.

Main Results:

  • HDAC6 is crucial for RMS tumor growth, self-renewal, migration, and invasion.
  • HDAC6 inhibition phenocopies loss-of-function effects.
  • HDAC6 regulates cytoskeletal dynamics, promoting RMS cell migration.
  • RAC1 is essential for HDAC6-mediated RMS cell migration and invasion.
  • High RAC1 expression correlates with poor clinical prognosis in RMS patients.

Conclusions:

  • The HDAC6-RAC1 axis is a key driver of RMS progression.
  • Targeting this axis presents a promising therapeutic strategy for improving RMS patient survival.

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