PMID- 30110925
OWN - NLM
STAT- MEDLINE
DCOM- 20181114
LR  - 20181202
IS  - 1422-0067 (Electronic)
IS  - 1422-0067 (Linking)
VI  - 19
IP  - 8
DP  - 2018 Aug 14
TI  - 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.
LID - 10.3390/ijms19082393 [doi]
LID - 2393
AB  - 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.
FAU - Park, Saeyoung
AU  - Park S
AD  - Department of Biochemistry, College of Medicine, Ewha Womans University, Seoul 
      07985, Korea. saeyoung@ewha.ac.kr.
FAU - Jung, Namhee
AU  - Jung N
AD  - Department of Biochemistry, College of Medicine, Ewha Womans University, Seoul 
      07985, Korea. polypola@ewhain.net.
FAU - Myung, Seoha
AU  - Myung S
AD  - Department of Biochemistry, College of Medicine, Ewha Womans University, Seoul 
      07985, Korea. myothink@naver.com.
FAU - Choi, Yoonyoung
AU  - Choi Y
AD  - Department of Biochemistry, College of Medicine, Ewha Womans University, Seoul 
      07985, Korea. yychoi27@ewhain.net.
FAU - Chung, Ki Wha
AU  - Chung KW
AD  - Department of Biological Sciences, Kongju National University, Gongju 32588, 
      Korea. kwchung@kongju.ac.kr.
FAU - Choi, Byung-Ok
AU  - Choi BO
AD  - Department of Neurology, Samsung Medical Center, Sungkyunkwan University School 
      of Medicine, Seoul 06351, Korea. bochoi77@hanmail.net.
FAU - Jung, Sung-Chul
AU  - Jung SC
AUID- ORCID: 0000-0002-3174-8965
AD  - Department of Biochemistry, College of Medicine, Ewha Womans University, Seoul 
      07985, Korea. jungsc@ewha.ac.kr.
LA  - eng
PT  - Journal Article
DEP - 20180814
PL  - Switzerland
TA  - Int J Mol Sci
JT  - International journal of molecular sciences
JID - 101092791
SB  - IM
MH  - Animals
MH  - *Cell Differentiation
MH  - Charcot-Marie-Tooth Disease/genetics/metabolism/pathology/*therapy
MH  - Disease Models, Animal
MH  - Heterografts
MH  - Humans
MH  - Male
MH  - Mesenchymal Stem Cells/*metabolism/pathology
MH  - Mice
MH  - Mice, Mutant Strains
MH  - Palatine Tonsil/*metabolism/pathology
MH  - *Recovery of Function
MH  - Schwann Cells/metabolism/pathology/*transplantation
PMC - PMC6121309
OTO - NOTNLM
OT  - Charcot-Marie-Tooth disease type 1A
OT  - Schwann cells
OT  - neuromuscular regeneration
OT  - remyelination
OT  - tonsil-derived mesenchymal stem cells
COIS- The authors declare no conflicts of interest.
EDAT- 2018/08/17 06:00
MHDA- 2018/11/15 06:00
PMCR- 2018/08/01
CRDT- 2018/08/17 06:00
PHST- 2018/06/21 00:00 [received]
PHST- 2018/08/06 00:00 [revised]
PHST- 2018/08/10 00:00 [accepted]
PHST- 2018/08/17 06:00 [entrez]
PHST- 2018/08/17 06:00 [pubmed]
PHST- 2018/11/15 06:00 [medline]
PHST- 2018/08/01 00:00 [pmc-release]
AID - ijms19082393 [pii]
AID - ijms-19-02393 [pii]
AID - 10.3390/ijms19082393 [doi]
PST - epublish
SO  - Int J Mol Sci. 2018 Aug 14;19(8):2393. doi: 10.3390/ijms19082393.