Comparison of neuromuscular junction of two kinds of mouse models of Duchenne muscular dystrophy

doi:10.3969/j.issn.2095-4344.2016.05.009

Chinese Journal of Tissue Engineering Research ›› 2016, Vol. 20 ›› Issue (5): 657-663.doi: 10.3969/j.issn.2095-4344.2016.05.009

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Comparison of neuromuscular junction of two kinds of mouse models of Duchenne muscular dystrophy

Kong Jie^{1, 2}, Cao Ji-qing¹, Yang Juan¹, Chen Fei¹, Li Ya-qin¹, Zhang Cheng¹

¹Department of Neurology, First Affiliated Hospital of Sun Yat-sen University, Guangzhou 510080, Guangdong Province, China; ²Department of Encephalopathy, Baoan District Hospital of Traditional Chinese Medicine, Guangzhou University of Chinese Medicine, Shenzhen 518133, Guangdong Province, China

Received:2015-11-29 Online:2016-01-29 Published:2016-01-29
About author:Kong Jie, M.D., Attending physician, Department of Neurology, First Affiliated Hospital of Sun Yat-sen University, Guangzhou 510080, Guangdong Province, China; Department of Encephalopathy, Baoan District Hospital of Traditional Chinese Medicine, Guangzhou University of Chinese Medicine, Shenzhen 518133, Guangdong Province, China
Supported by:
the Health and Family Planning System Research Project in 2015, No.201505021

Abstract

Abstract:

BACKGROUND: Neuromuscular junction structure has defects in patients with Duchenne muscular dystrophy, mainly presenting acetylcholine receptor structure fragmentation and the reduction of spine-like processes on the
postsynaptic membrane. It is generally recognized that the structural defects are induced by structural damage of muscle cells and muscle fiber necrosis.
OBJECTIVE: To explore the reasons of damage on neuromuscular junction in mouse models of Duchenne muscular dystrophy.
METHODS: We introduced Duchenne muscular dystrophy models of male mdx mice and male Dko mice. After gene identification, they were used for tests. Male C57BL/6 mice were selected as normla controls. Hematoxylin-eosin staining was utilized to detect pathological changes in muscles. Neuromuscular junction structure was revealed using immunofluorescence staining. The differences in dystrophin expression, pathological features and neuromuscular junction structure were compared in mouse models of two kinds of Duchenne muscular dystrophy.
RESULTS AND CONCLUSION: The introduced mouse models were accorded with the requirement of our experiment in aspects of genotype and protein expression levels. The number of acetylcholine receptor was apparently reduced in the neuromuscular junction of two kinds of mouse models. Although dko mouse muscles presented more obvious inflammatory infiltration and muscle fiber damage compared with mdx mice, but there was no significant difference in the damage to neuromuscular junction between them, and acetylcholine receptor fragmentation was identical. The evidence suggested that structural damage of neuromuscular junction and inflammatory pathological response are independent events. There is no direct relationship between them. Dystrophin gene deficiency is the main cause of the fragmentation of the acetylcholine receptor.

中国组织工程研究杂志出版内容重点：肾移植；肝移植；移植；心脏移植；组织移植；皮肤移植；皮瓣移植；血管移植；器官移植；组织工程

Kong Jie, Cao Ji-qing, Yang Juan, Chen Fei, Li Ya-qin, Zhang Cheng. Comparison of neuromuscular junction of two kinds of mouse models of Duchenne muscular dystrophy[J]. Chinese Journal of Tissue Engineering Research, 2016, 20(5): 657-663.

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References

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Comparison of neuromuscular junction of two kinds of mouse models of Duchenne muscular dystrophy

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