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Muscle Nerve. 2018 Mar;57(3):478-486. doi: 10.1002/mus.25763. Epub 2017 Aug 23.

Application of differentiated human tonsil-derived stem cells to trembler-J mice.

Author information

1
Department of Biochemistry, College of Medicine, Ewha Womans University, 1071 Anyangcheon-Ro, Yangcheon-Gu, Seoul, 07985, Republic of Korea.
2
Department of Health Sciences and Technology, Sungkyunkwan University, Seoul, Republic of Korea.
3
Stem Cell & Regenerative Medicine Institute, Samsung Medical Center, Seoul, Republic of Korea.
4
Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea.

Abstract

INTRODUCTION:

Mesenchymal stem cells (MSCs) can differentiate into various cell types.

METHODS:

In this study we investigated the potential of human tonsil-derived MSCs (T-MSCs) for neuromuscular regeneration in trembler-J (Tr-J) mice, a model for Charcot-Marie-Tooth disease type 1A (CMT1A).

RESULTS:

T-MSCs differentiated toward skeletal myocytes with increased expression of skeletal muscle-related markers (including troponin I type 1, and myogenin), and the formation of myotubes in vitro. In-situ transplantation of T-MSC-derived myocytes (T-MSC myocytes) into the gastrocnemius muscle in Tr-J mice enhanced motor function, with recovery of compound muscle action potential amplitudes. Morphology of the sciatic nerve and skeletal muscle recovered without the formation of teratomas, and the expression levels of nerve growth factor and glial-cell-line-derived neurotrophic factor were increased significantly in T-MSC myocytes compared with T-MSCs in vitro.

DISCUSSION:

Transplantation of T-MSC myocytes could enable neuromuscular regeneration in patients with CMT1A. Muscle Nerve 57: 478-486, 2018.

KEYWORDS:

Charcot-Marie-Tooth disease; neuromuscular; regeneration; stem cell therapy; tonsil-derived MSCs; trembler-J mice

PMID:
28796340
DOI:
10.1002/mus.25763
[Indexed for MEDLINE]

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