AI Article Synopsis
- Duchenne muscular dystrophy (DMD) is a condition characterized by the absence of dystrophin, leading to weak muscles, and muscle stem cell (MuSC) transplantation shows promise as a potential therapy.
- GFP-expressing MuSCs were transplanted into mice with DMD, and after three weeks, the neuromuscular junctions were examined to assess muscle fiber innervation.
- Results indicated that donor-derived muscle fibers had better structural innervation and showed signs of reinnervation, suggesting these fibers might integrate well into the damaged muscle environment.
Article Abstract
Introduction: Duchenne muscular dystrophy (DMD) is caused by loss of the structural protein, dystrophin, resulting in muscle fragility. Muscle stem cell (MuSC) transplantation is a potential therapy for DMD. It is unknown whether donor-derived muscle fibers are structurally innervated.
Methods: Green fluorescent protein (GFP)-expressing MuSCs were transplanted into the tibials anterior of adult dystrophic mdx/mTR mice. Three weeks later the neuromuscular junction was labeled by immunohistochemistry.
Results: The percent overlap between pre- and postsynaptic immunolabeling was greater in donor-derived GFP(+) myofibers, and fewer GFP(+) myofibers were identified as denervated compared with control GFP(-) fibers (P = 0.001 and 0.03). GFP(+) fibers also demonstrated acetylcholine receptor fragmentation and expanded endplate area, indicators of muscle reinnervation (P = 0.008 and 0.033).
Conclusion: It is unclear whether GFP(+) fibers are a result of de novo synthesis or fusion with damaged endogenous fibers. Either way, donor-derived fibers demonstrate clear histological innervation. Muscle Nerve 54: 763-768, 2016.
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Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5224835 | PMC |
| http://dx.doi.org/10.1002/mus.25115 | DOI Listing |
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