Satellite Cells in Muscular Dystrophy - Lost in Polarity

Copyright © 2016 Elsevier Ltd. All rights reserved..

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.

Medienart:

E-Artikel

Erscheinungsjahr:

2016

Erschienen:

2016

Enthalten in:

Zur Gesamtaufnahme - volume:22

Enthalten in:

Trends in molecular medicine - 22(2016), 6 vom: 10. Juni, Seite 479-496

Sprache:

Englisch

Beteiligte Personen:

Chang, Natasha C [VerfasserIn]
Chevalier, Fabien P [VerfasserIn]
Rudnicki, Michael A [VerfasserIn]

Links:

Volltext

Themen:

Asymmetric division
Duchenne muscular dystrophy
Dystrophin
Journal Article
Mark2
Par1b
Pard3
Regenerative myogenesis
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Review
Satellite cells
Stem cell polarity

Anmerkungen:

Date Completed 30.06.2017

Date Revised 11.01.2019

published: Print-Electronic

Citation Status MEDLINE

doi:

10.1016/j.molmed.2016.04.002

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM26018361X