MyoD is essential in rhabdomyosarcoma by promoting survival through differentiation and CYLD

Summary: Rhabdomyosarcoma (RMS) is the most common soft tissue cancer among children, characterized by a skeletal muscle lineage that is impaired from undergoing terminal differentiation. NF-κB is constitutively active in cancer cells and plays a critical role in cell survival. Although NF-κB is als...

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Main Authors: Alexander R. Oles, Peter Y. Yu, Abasi-ama Udeme, Sudarshana Sharma, Priya Londhe, Benjamin R. Pryce, Erin E. Talbert, Eric M. Hill, Carlos J. Miranda, Brian K. Kaspar, Michael A. Arnold, Jack Hyland, Cheryl A. London, Peter J. Houghton, David J. Wang, Ryan D. Roberts, Denis C. Guttridge
Format: Article
Language:English
Published: Elsevier 2025-08-01
Series:iScience
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Online Access:http://www.sciencedirect.com/science/article/pii/S2589004225014105
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author Alexander R. Oles
Peter Y. Yu
Abasi-ama Udeme
Sudarshana Sharma
Priya Londhe
Benjamin R. Pryce
Erin E. Talbert
Eric M. Hill
Carlos J. Miranda
Brian K. Kaspar
Michael A. Arnold
Jack Hyland
Cheryl A. London
Peter J. Houghton
David J. Wang
Ryan D. Roberts
Denis C. Guttridge
author_facet Alexander R. Oles
Peter Y. Yu
Abasi-ama Udeme
Sudarshana Sharma
Priya Londhe
Benjamin R. Pryce
Erin E. Talbert
Eric M. Hill
Carlos J. Miranda
Brian K. Kaspar
Michael A. Arnold
Jack Hyland
Cheryl A. London
Peter J. Houghton
David J. Wang
Ryan D. Roberts
Denis C. Guttridge
author_sort Alexander R. Oles
collection DOAJ
description Summary: Rhabdomyosarcoma (RMS) is the most common soft tissue cancer among children, characterized by a skeletal muscle lineage that is impaired from undergoing terminal differentiation. NF-κB is constitutively active in cancer cells and plays a critical role in cell survival. Although NF-κB is also activated in RMS, surprisingly, we find that these tumors are far less dependent on NF-κB for their survival. Instead, RMS cells survive, paradoxically, by being partially differentiated under the control of the myogenic transcription factor MyoD. Loss of MyoD, or cellular reprogramming, dedifferentiates RMS tumor cells and sensitizes their death under stress. MyoD enhances RMS survival by regulating DNA methyltransferases, which in turn suppresses the tumor suppressor and pro-apoptotic gene CYLD. From these findings, we propose that MyoD acts as an oncogene in RMS by enhancing survival through pro-differentiation and anti-cell death activities.
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publishDate 2025-08-01
publisher Elsevier
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series iScience
spelling doaj-art-e93de17eabd348f5b7cfc68f76f35bca2025-08-20T03:22:39ZengElsevieriScience2589-00422025-08-0128811314910.1016/j.isci.2025.113149MyoD is essential in rhabdomyosarcoma by promoting survival through differentiation and CYLDAlexander R. Oles0Peter Y. Yu1Abasi-ama Udeme2Sudarshana Sharma3Priya Londhe4Benjamin R. Pryce5Erin E. Talbert6Eric M. Hill7Carlos J. Miranda8Brian K. Kaspar9Michael A. Arnold10Jack Hyland11Cheryl A. London12Peter J. Houghton13David J. Wang14Ryan D. Roberts15Denis C. Guttridge16Department of Pediatrics, Darby Children’s Research Institute, Medical University of South Carolina, Charleston, SC 29425, USADepartment of Cancer Biology and Genetics, The Ohio State University Medical Center, Columbus, OH 43210, USADepartment of Pediatrics, Darby Children’s Research Institute, Medical University of South Carolina, Charleston, SC 29425, USAHollings Cancer Center, Medical University of South Carolina, Charleston, SC 29425, USADepartment of Cancer Biology and Genetics, The Ohio State University Medical Center, Columbus, OH 43210, USADepartment of Pediatrics, Darby Children’s Research Institute, Medical University of South Carolina, Charleston, SC 29425, USADepartment of Health and Human Physiology, and the Holden Comprehensive Cancer Center, University of Iowa, Iowa City, IA 52242, USADepartment of Cancer Biology and Genetics, The Ohio State University Medical Center, Columbus, OH 43210, USADepartment of Pediatrics, Center for Gene Therapy, Nationwide Children’s Hospital, Columbus, OH 43205, USADepartment of Pediatrics, Center for Gene Therapy, Nationwide Children’s Hospital, Columbus, OH 43205, USADepartment of Pathology and Laboratory Medicine, Children’s Hospital Colorado, Aurora, CO 80045, USA; Department of Pathology and Laboratory Medicine, University of Colorado, Anschutz Medical Campus, Aurora, CO 80045, USADepartment of Pediatrics, Darby Children’s Research Institute, Medical University of South Carolina, Charleston, SC 29425, USACummings School of Veterinary Medicine, Tufts University, Medford, MA 02155, USAGreehey Children’s Cancer Research Institute, University of Texas, San Antonio, TX 23220, USADepartment of Pediatrics, Darby Children’s Research Institute, Medical University of South Carolina, Charleston, SC 29425, USADepartment of Pediatrics, Center for Childhood Cancer, Nationwide Children’s Hospital, Columbus, OH 43205, USADepartment of Pediatrics, Darby Children’s Research Institute, Medical University of South Carolina, Charleston, SC 29425, USA; Hollings Cancer Center, Medical University of South Carolina, Charleston, SC 29425, USA; Corresponding authorSummary: Rhabdomyosarcoma (RMS) is the most common soft tissue cancer among children, characterized by a skeletal muscle lineage that is impaired from undergoing terminal differentiation. NF-κB is constitutively active in cancer cells and plays a critical role in cell survival. Although NF-κB is also activated in RMS, surprisingly, we find that these tumors are far less dependent on NF-κB for their survival. Instead, RMS cells survive, paradoxically, by being partially differentiated under the control of the myogenic transcription factor MyoD. Loss of MyoD, or cellular reprogramming, dedifferentiates RMS tumor cells and sensitizes their death under stress. MyoD enhances RMS survival by regulating DNA methyltransferases, which in turn suppresses the tumor suppressor and pro-apoptotic gene CYLD. From these findings, we propose that MyoD acts as an oncogene in RMS by enhancing survival through pro-differentiation and anti-cell death activities.http://www.sciencedirect.com/science/article/pii/S2589004225014105Cell biologyCancer
spellingShingle Alexander R. Oles
Peter Y. Yu
Abasi-ama Udeme
Sudarshana Sharma
Priya Londhe
Benjamin R. Pryce
Erin E. Talbert
Eric M. Hill
Carlos J. Miranda
Brian K. Kaspar
Michael A. Arnold
Jack Hyland
Cheryl A. London
Peter J. Houghton
David J. Wang
Ryan D. Roberts
Denis C. Guttridge
MyoD is essential in rhabdomyosarcoma by promoting survival through differentiation and CYLD
iScience
Cell biology
Cancer
title MyoD is essential in rhabdomyosarcoma by promoting survival through differentiation and CYLD
title_full MyoD is essential in rhabdomyosarcoma by promoting survival through differentiation and CYLD
title_fullStr MyoD is essential in rhabdomyosarcoma by promoting survival through differentiation and CYLD
title_full_unstemmed MyoD is essential in rhabdomyosarcoma by promoting survival through differentiation and CYLD
title_short MyoD is essential in rhabdomyosarcoma by promoting survival through differentiation and CYLD
title_sort myod is essential in rhabdomyosarcoma by promoting survival through differentiation and cyld
topic Cell biology
Cancer
url http://www.sciencedirect.com/science/article/pii/S2589004225014105
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