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Differentiation of human tonsil-derived mesenchymal stem cells into Schwann-like cells improves neuromuscular function in a mouse model of Charcot-Marie-Tooth disease type 1A

Title
Differentiation of human tonsil-derived mesenchymal stem cells into Schwann-like cells improves neuromuscular function in a mouse model of Charcot-Marie-Tooth disease type 1A
Authors
Park S.Jung N.Myung S.Choi Y.Chung K.W.Choi B.-O.Jung S.-C.
Ewha Authors
정성철
SCOPUS Author ID
정성철scopus
Issue Date
2018
Journal Title
International Journal of Molecular Sciences
ISSN
1661-6596JCR Link
Citation
vol. 19, no. 8
Keywords
Charcot-Marie-Tooth disease type 1aNeuromuscular regenerationRemyelinationSchwann cellsTonsil-derived mesenchymal stem cells
Publisher
MDPI AG
Indexed
SCOPUS scopus
Abstract
Charcot-Marie-Tooth disease type 1A (CMT1A) is the most common inherited motor and sensory neuropathy, and is caused by duplication of PMP22, alterations of which are a characteristic feature of demyelination. The clinical phenotype of CMT1A is determined by the degree of axonal loss, and patients suffer from progressive muscle weakness and impaired sensation. Therefore, we investigated the potential of Schwann-like cells differentiated from human tonsil-derived stem cells (T-MSCs) for use in neuromuscular regeneration in trembler-J (Tr-J) mice, a model of CMT1A. After differentiation, we confirmed the increased expression of Schwann cell (SC) markers, including glial fibrillary acidic protein (GFAP), nerve growth factor receptor (NGFR), S100 calcium-binding protein B (S100B), glial cell-derived neurotrophic factor (GDNF), and brain-derived neurotrophic factor (BDNF), which suggests the differentiation of T-MSCs into SCs (T-MSC-SCs). To test their functional efficiency, the T-MSC-SCs were transplanted into the caudal thigh muscle of Tr-J mice. Recipients’ improved locomotive activity on a rotarod test, and their sciatic function index, which suggests that transplanted T-MSC-SCs ameliorated demyelination and atrophy of nerve and muscle in Tr-J mice. Histological and molecular analyses showed the possibility of in situ remyelination by T-MSC-SCs transplantation. These findings demonstrate that the transplantation of heterologous T-MSC-SCs induced neuromuscular regeneration in mice and suggest they could be useful for the therapeutic treatment of patients with CMT1A disease. © 2018 by the authors. Licensee MDPI, Basel, Switzerland.
DOI
10.3390/ijms19082393
Appears in Collections:
의과대학 > 의학과 > Journal papers
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