Satellite cells of skeletal muscle fibers in human progressive muscular dystrophy

1963 ◽  
Vol 336 (6) ◽  
pp. 564-569 ◽  
Author(s):  
Ruben Laguens
Keyword(s):  
Skeletal Muscle ◽  
Muscular Dystrophy ◽  
Satellite Cells ◽  
Muscle Fibers ◽  
Progressive Muscular Dystrophy ◽  
Skeletal Muscle Fibers

scholarly journals Multilineage Differentiation for Formation of Innervated Skeletal Muscle Fibers from Healthy and Diseased Human Pluripotent Stem Cells

Cells ◽  
2020 ◽  
Vol 9 (6) ◽  
pp. 1531 ◽  
Author(s):  
Kilian Mazaleyrat ◽  
Cherif Badja ◽  
Natacha Broucqsault ◽  
Raphaël Chevalier ◽  
Camille Laberthonnière ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Stem Cells ◽  
Muscular Dystrophy ◽  
Pluripotent Stem Cells ◽  
Cell Biology ◽  
Disease Modeling ◽  
Muscle Fibers ◽  
Functional Evaluation ◽  
Skeletal Muscle Fibers ◽  
Human Ipscs

Induced pluripotent stem cells (iPSCs) obtained by reprogramming primary somatic cells have revolutionized the fields of cell biology and disease modeling. However, the number protocols for generating mature muscle fibers with sarcolemmal organization using iPSCs remain limited, and partly mimic the complexity of mature skeletal muscle. Methods: We used a novel combination of small molecules added in a precise sequence for the simultaneous codifferentiation of human iPSCs into skeletal muscle cells and motor neurons. Results: We show that the presence of both cell types reduces the production time for millimeter-long multinucleated muscle fibers with sarcolemmal organization. Muscle fiber contractions are visible in 19–21 days, and can be maintained over long period thanks to the production of innervated multinucleated mature skeletal muscle fibers with autonomous cell regeneration of PAX7-positive cells and extracellular matrix synthesis. The sequential addition of specific molecules recapitulates key steps of human peripheral neurogenesis and myogenesis. Furthermore, this organoid-like culture can be used for functional evaluation and drug screening. Conclusion: Our protocol, which is applicable to hiPSCs from healthy individuals, was validated in Duchenne Muscular Dystrophy, Myotonic Dystrophy, Facio-Scapulo-Humeral Dystrophy and type 2A Limb-Girdle Muscular Dystrophy, opening new paths for the exploration of muscle differentiation, disease modeling and drug discovery.

scholarly journals MyoD protein accumulates in satellite cells and is neurally regulated in regenerating myotubes and skeletal muscle fibers

1995 ◽  
Vol 202 (3) ◽  
pp. 244-254 ◽  
Author(s):  
Kyoko Koishi ◽  
Ming Zhang ◽  
Ian S. McLennan ◽  
A. John Harris
Keyword(s):  
Skeletal Muscle ◽  
Satellite Cells ◽  
Muscle Fibers ◽  
Skeletal Muscle Fibers
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