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Muscle stem cell dysfunction in rhabdomyosarcoma and muscular dystrophy.
Robertson, Rebecca; Li, Shulei; Filippelli, Romina L; Chang, Natasha C.
Afiliação
  • Robertson R; Department of Biochemistry, Faculty of Medicine and Health Sciences, McGill University, Montréal, QC, Canada.
  • Li S; Department of Biochemistry, Faculty of Medicine and Health Sciences, McGill University, Montréal, QC, Canada; Rosalind and Morris Goodman Cancer Institute, McGill University, Montréal, QC, Canada.
  • Filippelli RL; Department of Biochemistry, Faculty of Medicine and Health Sciences, McGill University, Montréal, QC, Canada.
  • Chang NC; Department of Biochemistry, Faculty of Medicine and Health Sciences, McGill University, Montréal, QC, Canada; Rosalind and Morris Goodman Cancer Institute, McGill University, Montréal, QC, Canada. Electronic address: natasha.chang@mcgill.ca.
Curr Top Dev Biol ; 158: 83-121, 2024.
Article em En | MEDLINE | ID: mdl-38670717
ABSTRACT
Muscle stem cells (MuSCs) are crucial to the repair and homeostasis of mature skeletal muscle. MuSC dysfunction and dysregulation of the myogenic program can contribute to the development of pathology ranging from cancers like rhabdomyosarcoma (RMS) or muscle degenerative diseases such as Duchenne muscular dystrophy (DMD). Both diseases exhibit dysregulation at nearly all steps of myogenesis. For instance, MuSC self-renewal processes are altered. In RMS, this leads to the creation of tumor propagating cells. In DMD, impaired asymmetric stem cell division creates a bias towards producing self-renewing stem cells instead of committing to differentiation. Hyperproliferation of these cells contribute to tumorigenesis in RMS and symmetric expansion of the self-renewing MuSC population in DMD. Both diseases also exhibit a repression of factors involved in terminal differentiation, halting RMS cells in the proliferative stage and thus driving tumor growth. Conversely, the MuSCs in DMD exhibit impaired differentiation and fuse prematurely, affecting myonuclei maturation and the integrity of the dystrophic muscle fiber. Finally, both disease states cause alterations to the MuSC niche. Various elements of the niche such as inflammatory and migratory signaling that impact MuSC behavior are dysregulated. Here we show how these seemingly distantly related diseases indeed have similarities in MuSC dysfunction, underlying the importance of considering MuSCs when studying the pathophysiology of muscle diseases.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Rabdomiossarcoma Limite: Animals / Humans Idioma: En Revista: Curr Top Dev Biol Ano de publicação: 2024 Tipo de documento: Article País de afiliação: Canadá

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Rabdomiossarcoma Limite: Animals / Humans Idioma: En Revista: Curr Top Dev Biol Ano de publicação: 2024 Tipo de documento: Article País de afiliação: Canadá