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Induced Pluripotent Stem Cells derived Muscle Progenitors Effectively Mitigate Muscular Dystrophy through Restoring the Dystrophin Distribution.
J Stem Cell Res Ther. 2016; 6(10)JS

Abstract

BACKGROUND

Duchenne Muscular Dystrophy (DMD) is a recessive form of muscular disorder, resulting from the dystrophin gene mutations in X-chromosome. Application of embryonic stem cells or adult stem cells has demonstrated the therapeutic effects on DMD through both cell-based and non-cell based mechanisms. In this study, we proposed that Myogenic Progenitor Cells from Induced Pluripotent Stem Cells (iPSC-MPCs) would be more effective in repairing muscle damage caused by muscular dystrophy.

METHODS AND RESULTS

Mouse iPSCs were cultured in myogenic differentiation culture medium and the MPCs were characterized using Reverse Transcription Polymerase Chain Reaction (RT-PCR) and flow cytometry. iPSCs were successfully converted into MPCs, as evidenced by the distinct expression of myogenic genes and cell surface markers. The muscle injury was induced in tibialis muscle of mdx mouse by cardiotoxin injection, and the iPSC-MPCs were then engrafted into the damage site. Firefly luciferase expression vector was transduced into iPSC-MPCs and the in vivo bioluminescence imaging analysis revealed that these progenitor cells survived even at 30-days post transplantation. Importantly, histological analysis revealed that the central nuclei percentage, as well as fibrosis, was significantly reduced in the iPSC-MPCs treated muscle. In addition,the transplantation of progenitor cells restored the distributions of dystrophin and nicotinic acetylcholine receptors together with up-regulation of pair box protein 7(Pax7), a myogenic transcription factor.

CONCLUSION

iPSCs-derived MPCs exert strong therapeutic effects on muscular dystrophy by restoring dystrophin expression and acetylcholine receptor distribution.

Authors+Show Affiliations

Department of Pathology and Lab Medicine, College of Medicine, University of Cincinnati, Cincinnati, OH 45267-0529, USA.Department of Pathology and Lab Medicine, College of Medicine, University of Cincinnati, Cincinnati, OH 45267-0529, USA.Department of Pathology and Lab Medicine, College of Medicine, University of Cincinnati, Cincinnati, OH 45267-0529, USA.Department of Pathology and Lab Medicine, College of Medicine, University of Cincinnati, Cincinnati, OH 45267-0529, USA.Department of Pathology and Lab Medicine, College of Medicine, University of Cincinnati, Cincinnati, OH 45267-0529, USA. Key Laboratory of Functional Proteomics of Guangdong Province, Department of Pathophysiology, Southern Medical University, Guangzhou 510515, China.Department of Pharmacology, University of Illinois at Chicago, Chicago, IL 60612, USA.Qinghai Provincial People's Hospital, 2 Gonghe Rd, Xining, Qinghai, 810007, China.Department of Pathology and Lab Medicine, College of Medicine, University of Cincinnati, Cincinnati, OH 45267-0529, USA.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

28979820

Citation

Cai, Wen-Feng, et al. "Induced Pluripotent Stem Cells Derived Muscle Progenitors Effectively Mitigate Muscular Dystrophy Through Restoring the Dystrophin Distribution." Journal of Stem Cell Research & Therapy, vol. 6, no. 10, 2016.
Cai WF, Huang W, Wang L, et al. Induced Pluripotent Stem Cells derived Muscle Progenitors Effectively Mitigate Muscular Dystrophy through Restoring the Dystrophin Distribution. J Stem Cell Res Ther. 2016;6(10).
Cai, W. F., Huang, W., Wang, L., Wang, J. P., Zhang, L., Ashraf, M., Wu, S., & Wang, Y. (2016). Induced Pluripotent Stem Cells derived Muscle Progenitors Effectively Mitigate Muscular Dystrophy through Restoring the Dystrophin Distribution. Journal of Stem Cell Research & Therapy, 6(10). https://doi.org/10.4172/2157-7633.1000361
Cai WF, et al. Induced Pluripotent Stem Cells Derived Muscle Progenitors Effectively Mitigate Muscular Dystrophy Through Restoring the Dystrophin Distribution. J Stem Cell Res Ther. 2016;6(10) PubMed PMID: 28979820.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Induced Pluripotent Stem Cells derived Muscle Progenitors Effectively Mitigate Muscular Dystrophy through Restoring the Dystrophin Distribution. AU - Cai,Wen-Feng, AU - Huang,Wei, AU - Wang,Lei, AU - Wang,Jia-Peng, AU - Zhang,Lu, AU - Ashraf,Muhammad, AU - Wu,Shizheng, AU - Wang,Yigang, Y1 - 2016/09/26/ PY - 2017/10/6/entrez PY - 2016/1/1/pubmed PY - 2016/1/1/medline KW - Duchenne muscular dystrophy KW - Dystrophin KW - Muscular progenitors KW - iPSCs JF - Journal of stem cell research & therapy JO - J Stem Cell Res Ther VL - 6 IS - 10 N2 - BACKGROUND: Duchenne Muscular Dystrophy (DMD) is a recessive form of muscular disorder, resulting from the dystrophin gene mutations in X-chromosome. Application of embryonic stem cells or adult stem cells has demonstrated the therapeutic effects on DMD through both cell-based and non-cell based mechanisms. In this study, we proposed that Myogenic Progenitor Cells from Induced Pluripotent Stem Cells (iPSC-MPCs) would be more effective in repairing muscle damage caused by muscular dystrophy. METHODS AND RESULTS: Mouse iPSCs were cultured in myogenic differentiation culture medium and the MPCs were characterized using Reverse Transcription Polymerase Chain Reaction (RT-PCR) and flow cytometry. iPSCs were successfully converted into MPCs, as evidenced by the distinct expression of myogenic genes and cell surface markers. The muscle injury was induced in tibialis muscle of mdx mouse by cardiotoxin injection, and the iPSC-MPCs were then engrafted into the damage site. Firefly luciferase expression vector was transduced into iPSC-MPCs and the in vivo bioluminescence imaging analysis revealed that these progenitor cells survived even at 30-days post transplantation. Importantly, histological analysis revealed that the central nuclei percentage, as well as fibrosis, was significantly reduced in the iPSC-MPCs treated muscle. In addition,the transplantation of progenitor cells restored the distributions of dystrophin and nicotinic acetylcholine receptors together with up-regulation of pair box protein 7(Pax7), a myogenic transcription factor. CONCLUSION: iPSCs-derived MPCs exert strong therapeutic effects on muscular dystrophy by restoring dystrophin expression and acetylcholine receptor distribution. SN - 2157-7633 UR - https://www.unboundmedicine.com/medline/citation/28979820/Induced_Pluripotent_Stem_Cells_derived_Muscle_Progenitors_Effectively_Mitigate_Muscular_Dystrophy_through_Restoring_the_Dystrophin_Distribution_ L2 - https://www.ncbi.nlm.nih.gov/pmc/articles/pmid/28979820/ DB - PRIME DP - Unbound Medicine ER -
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