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Satellite Cells in Muscular Dystrophy - Lost in Polarity.
Trends Mol Med. 2016 06; 22(6):479-496.TM

Abstract

Recent findings employing the mdx mouse model for Duchenne muscular dystrophy (DMD) have revealed that muscle satellite stem cells play a direct role in contributing to disease etiology and progression of DMD, the most common and severe form of muscular dystrophy. Lack of dystrophin expression in DMD has critical consequences in satellite cells including an inability to establish cell polarity, abrogation of asymmetric satellite stem-cell divisions, and failure to enter the myogenic program. Thus, muscle wasting in dystrophic mice is not only caused by myofiber fragility but is exacerbated by intrinsic satellite cell dysfunction leading to impaired regeneration. Despite intense research and clinical efforts, there is still no effective cure for DMD. In this review we highlight recent research advances in DMD and discuss the current state of treatment and, importantly, how we can incorporate satellite cell-targeted therapeutic strategies to correct satellite cell dysfunction in DMD.

Authors+Show Affiliations

Sprott Centre for Stem Cell Research, Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, ON, K1H 8L6, Canada; Department of Cellular and Molecular Medicine, Faculty of Medicine, University of Ottawa, Ottawa, ON, K1H 8M5, Canada.Sprott Centre for Stem Cell Research, Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, ON, K1H 8L6, Canada; Department of Cellular and Molecular Medicine, Faculty of Medicine, University of Ottawa, Ottawa, ON, K1H 8M5, Canada.Sprott Centre for Stem Cell Research, Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, ON, K1H 8L6, Canada; Department of Cellular and Molecular Medicine, Faculty of Medicine, University of Ottawa, Ottawa, ON, K1H 8M5, Canada. Electronic address: mrudnicki@ohri.ca.

Pub Type(s)

Journal Article
Review
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

27161598

Citation

Chang, Natasha C., et al. "Satellite Cells in Muscular Dystrophy - Lost in Polarity." Trends in Molecular Medicine, vol. 22, no. 6, 2016, pp. 479-496.
Chang NC, Chevalier FP, Rudnicki MA. Satellite Cells in Muscular Dystrophy - Lost in Polarity. Trends Mol Med. 2016;22(6):479-496.
Chang, N. C., Chevalier, F. P., & Rudnicki, M. A. (2016). Satellite Cells in Muscular Dystrophy - Lost in Polarity. Trends in Molecular Medicine, 22(6), 479-496. https://doi.org/10.1016/j.molmed.2016.04.002
Chang NC, Chevalier FP, Rudnicki MA. Satellite Cells in Muscular Dystrophy - Lost in Polarity. Trends Mol Med. 2016;22(6):479-496. PubMed PMID: 27161598.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Satellite Cells in Muscular Dystrophy - Lost in Polarity. AU - Chang,Natasha C, AU - Chevalier,Fabien P, AU - Rudnicki,Michael A, Y1 - 2016/05/05/ PY - 2016/03/25/received PY - 2016/04/13/revised PY - 2016/04/14/accepted PY - 2016/5/11/entrez PY - 2016/5/11/pubmed PY - 2017/7/1/medline KW - Duchenne muscular dystrophy KW - Mark2 KW - Par1b KW - Pard3 KW - asymmetric division KW - dystrophin KW - regenerative myogenesis KW - satellite cells KW - stem cell polarity SP - 479 EP - 496 JF - Trends in molecular medicine JO - Trends Mol Med VL - 22 IS - 6 N2 - Recent findings employing the mdx mouse model for Duchenne muscular dystrophy (DMD) have revealed that muscle satellite stem cells play a direct role in contributing to disease etiology and progression of DMD, the most common and severe form of muscular dystrophy. Lack of dystrophin expression in DMD has critical consequences in satellite cells including an inability to establish cell polarity, abrogation of asymmetric satellite stem-cell divisions, and failure to enter the myogenic program. Thus, muscle wasting in dystrophic mice is not only caused by myofiber fragility but is exacerbated by intrinsic satellite cell dysfunction leading to impaired regeneration. Despite intense research and clinical efforts, there is still no effective cure for DMD. In this review we highlight recent research advances in DMD and discuss the current state of treatment and, importantly, how we can incorporate satellite cell-targeted therapeutic strategies to correct satellite cell dysfunction in DMD. SN - 1471-499X UR - https://www.unboundmedicine.com/medline/citation/27161598/Satellite_Cells_in_Muscular_Dystrophy___Lost_in_Polarity_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1471-4914(16)30006-5 DB - PRIME DP - Unbound Medicine ER -