scholarly journals “The Social Network” and Muscular Dystrophies: The Lesson Learnt about the Niche Environment as a Target for Therapeutic Strategies

Cells ◽  
2020 ◽  
Vol 9 (7) ◽  
pp. 1659 ◽  
Author(s):  
Ornella Cappellari ◽  
Paola Mantuano ◽  
Annamaria De Luca
Keyword(s):  
Muscular Dystrophy ◽  
Satellite Cell ◽  
Cell Activation ◽  
Neuromuscular Disorders ◽  
Cell Loss ◽  
Regenerative Process ◽  
Muscle Stem Cells ◽  
Inhibitory Mechanisms ◽  
Muscle Necrosis ◽  
Satellite Cell Activation

The muscle stem cells niche is essential in neuromuscular disorders. Muscle injury and myofiber death are the main triggers of muscle regeneration via satellite cell activation. However, in degenerative diseases such as muscular dystrophy, regeneration still keep elusive. In these pathologies, stem cell loss occurs over time, and missing signals limiting damaged tissue from activating the regenerative process can be envisaged. It is unclear what comes first: the lack of regeneration due to satellite cell defects, their pool exhaustion for degeneration/regeneration cycles, or the inhibitory mechanisms caused by muscle damage and fibrosis mediators. Herein, Duchenne muscular dystrophy has been taken as a paradigm, as several drugs have been tested at the preclinical and clinical levels, targeting secondary events in the complex pathogenesis derived from lack of dystrophin. We focused on the crucial roles that pro-inflammatory and pro-fibrotic cytokines play in triggering muscle necrosis after damage and stimulating satellite cell activation and self-renewal, along with growth and mechanical factors. These processes contribute to regeneration and niche maintenance. We review the main effects of drugs on regeneration biomarkers to assess whether targeting pathogenic events can help to protect niche homeostasis and enhance regeneration efficiency other than protecting newly formed fibers from further damage.

scholarly journals Restoring the regenerative balance in neuromuscular disorders: satellite cell activation as therapeutic target in Pompe disease

2019 ◽  
Vol 7 (13) ◽  
pp. 280-280
Author(s):  
Gerben J. Schaaf ◽  
Rodrigo Canibano-Fraile ◽  
Tom J. M. van Gestel ◽  
Ans T. van der Ploeg ◽  
W. W. M. Pim Pijnappel
Keyword(s):  
Satellite Cell ◽  
Pompe Disease ◽  
Therapeutic Target ◽  
Cell Activation ◽  
Neuromuscular Disorders ◽  
Satellite Cell Activation

scholarly journals Lack of robust satellite cell activation and muscle regeneration during the progression of Pompe disease

2015 ◽  
Vol 3 (1) ◽  
Author(s):  
Gerben J. Schaaf ◽  
Tom JM van Gestel ◽  
Esther Brusse ◽  
Robert M. Verdijk ◽  
Irenaeus FM de Coo ◽  
...  
Keyword(s):  
Satellite Cell ◽  
Pompe Disease ◽  
Muscle Regeneration ◽  
Cell Activation ◽  
Satellite Cell Activation

scholarly journals Metformin Delays Satellite Cell Activation and Maintains Quiescence

2019 ◽  
Vol 2019 ◽  
pp. 1-19 ◽  
Author(s):  
Theodora Pavlidou ◽  
Milica Marinkovic ◽  
Marco Rosina ◽  
Claudia Fuoco ◽  
Simone Vumbaca ◽  
...  
Keyword(s):  
Satellite Cell ◽  
Muscle Tissue ◽  
Satellite Cells ◽  
Cell Activation ◽  
Myogenic Differentiation ◽  
Metabolic State ◽  
Cell Pool ◽  
Age Related ◽  
Satellite Cell Activation

The regeneration of the muscle tissue relies on the capacity of the satellite stem cell (SC) population to exit quiescence, divide asymmetrically, proliferate, and differentiate. In age-related muscle atrophy (sarcopenia) and several dystrophies, regeneration cannot compensate for the loss of muscle tissue. These disorders are associated with the depletion of the satellite cell pool or with the loss of satellite cell functionality. Recently, the establishment and maintenance of quiescence in satellite cells have been linked to their metabolic state. In this work, we aimed to modulate metabolism in order to preserve the satellite cell pool. We made use of metformin, a calorie restriction mimicking drug, to ask whether metformin has an effect on quiescence, proliferation, and differentiation of satellite cells. We report that satellite cells, when treated with metformin in vitro, ex vivo, or in vivo, delay activation, Pax7 downregulation, and terminal myogenic differentiation. We correlate the metformin-induced delay in satellite cell activation with the inhibition of the ribosome protein RPS6, one of the downstream effectors of the mTOR pathway. Moreover, in vivo administration of metformin induces a belated regeneration of cardiotoxin- (CTX-) damaged skeletal muscle. Interestingly, satellite cells treated with metformin immediately after isolation are smaller in size and exhibit reduced pyronin Y levels, which suggests that metformin-treated satellite cells are transcriptionally less active. Thus, our study suggests that metformin delays satellite cell activation and differentiation by favoring a quiescent, low metabolic state.

scholarly journals Therapeutic Strategies for Duchenne Muscular Dystrophy: An Update

Genes ◽  
2020 ◽  
Vol 11 (8) ◽  
pp. 837 ◽  
Author(s):  
Chengmei Sun ◽  
Luoan Shen ◽  
Zheng Zhang ◽  
Xin Xie
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Motor Neurons ◽  
Neuromuscular Junctions ◽  
Neuromuscular Disorders ◽  
Dystrophin Gene ◽  
Current Status ◽  
Muscle Stem Cells ◽  
Cell Based Therapy ◽  
Dystrophin Protein

Neuromuscular disorders encompass a heterogeneous group of conditions that impair the function of muscles, motor neurons, peripheral nerves, and neuromuscular junctions. Being the most common and most severe type of muscular dystrophy, Duchenne muscular dystrophy (DMD), is caused by mutations in the X-linked dystrophin gene. Loss of dystrophin protein leads to recurrent myofiber damage, chronic inflammation, progressive fibrosis, and dysfunction of muscle stem cells. Over the last few years, there has been considerable development of diagnosis and therapeutics for DMD, but current treatments do not cure the disease. Here, we review the current status of DMD pathogenesis and therapy, focusing on mutational spectrum, diagnosis tools, clinical trials, and therapeutic approaches including dystrophin restoration, gene therapy, and myogenic cell transplantation. Furthermore, we present the clinical potential of advanced strategies combining gene editing, cell-based therapy with tissue engineering for the treatment of muscular dystrophy.

scholarly journals Castration induces satellite cell activation that contributes to skeletal muscle maintenance

2018 ◽  
Vol 1 (1) ◽  
pp. 1-16 ◽  
Author(s):  
Alanna Klose ◽  
Wenxuan Liu ◽  
Nicole D. Paris ◽  
Sophie Forman ◽  
John J. Krolewski ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Satellite Cell ◽  
Cell Activation ◽  
Satellite Cell Activation

Satellite cell activation and retention of muscle regenerative potential after long-term denervation

Stem Cells ◽  
2020 ◽  
Vol 39 (3) ◽  
pp. 331-344
Author(s):  
Alvin Wong ◽  
Steven M. Garcia ◽  
Stanley Tamaki ◽  
Katharine Striedinger ◽  
Emilie Barruet ◽  
...  
Keyword(s):  
Satellite Cell ◽  
Cell Activation ◽  
Satellite Cell Activation

Satellite cell activation after muscle damage in young and adult rats

1995 ◽  
Vol 242 (3) ◽  
pp. 329-336 ◽  
Author(s):  
S. C. J. M. Jacobs ◽  
J. H. J. Wokke ◽  
P. R. Bär ◽  
A. L. Bootsma
Keyword(s):  
Muscle Damage ◽  
Satellite Cell ◽  
Cell Activation ◽  
Adult Rats ◽  
Satellite Cell Activation
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