Ts1Cje mouse model for Down syndrome exhibits motor function deficit; the potential role of skeletal muscles and peripheral nervous system
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1
Universiti Putra Malaysia, NeuroBiology and Genetics Group, Genetics and Regenerative Medicine Research Centre, Faculty of Medicine and Health Sciences, Malaysia
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2
Universiti Putra Malaysia, Department of Human Anatomy, Faculty of Medicine and Health Sciences, Malaysia
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3
Gombe State University, Department of Human Anatomy, College of Medical Sciences, Nigeria
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4
Universiti Putra Malaysia, Department of Pathology, Faculty of Medicine and Health Sciences, Malaysia
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5
Universiti Putra Malaysia, Medical Genetics Unit, Department of Biomedical Sciences, Faculty of Medicine and Health Sciences, Malaysia
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6
Universiti Putra Malaysia, Department of Community Health, Faculty of Medicine and Health Sciences, Malaysia
Down syndrome (DS) is a chromosomal abnormality caused by the presence of extra-copy of human chromosome 21. Motor dysfunction due to hypotonia is commonly seen in DS individuals and its etiology is still unknown. In this study, we employed the Ts1Cje, a mouse model for DS to investigate the motor performance in vivo and to elucidate the role of skeletal muscle and peripheral nervous system in causing hypotonia. Behavioral assessment of the motor performance showed that, the Ts1Cje mice exhibit weaker muscle strength. The automated grip test result revealed that forelimb strength of the wild type mice was significantly (P<0.0001) better than the Ts1Cje mice. The ability of the mice to survive the hanging position was also compared. The survival time of the wild type mice was significantly better (P<0.01) than the Ts1Cje mice. For motor coordination, the wild type mice performed significantly (P<0.01) better than the Ts1Cje mice. There was difference in the expression of the myogenic regulatory factors (MRFs), as the Myf5 was significantly down regulated while MyoD was upregulated in the Ts1Cje skeletal muscle. The expression level of genes involved in neuromuscular transmission, skeletal muscles structure and function was altered, but it was not statistically significant. Assessment of the myelin level revealed lower myelination in the sciatic nerve of the Ts1Cje mice but no morphological difference was observed between groups. Both adult and aging groups of the Ts1Cje mice further exhibited significantly (P<0.001) lower conduction velocity compared with their age-matching wild types. Metabolic activities, muscle fibre type and pathological changes in the skeletal muscle were further accessed using different staining techniques. We therefore conclude that the Ts1Cje mice exhibits muscle weakness and the alteration in MRFs expression together with low myelination in the sciatic nerve may contribute to motor dysfunction seen in Ts1Cje mice.
Acknowledgements
This work was supported by a Research University Grant Scheme (RUGS), Universiti Putra Malaysia (UPM) (04-02-12-2102RU) awarded to King-Hwa Ling; A Science Fund, Malaysian Ministry of Science, Technology and Innovation (MOSTI) (02-01-04-SF1306) and Exploratory Research Grant Scheme, Malaysian Ministry of Education (ERGS/1/11/SKK/UPM/03/1) was awarded to Pike-See Cheah.
Keywords:
Down Syndrome,
Gene Expression,
skeletal muscle,
in vivo,
hypotonia
Conference:
14th Meeting of the Asian-Pacific Society for Neurochemistry, Kuala Lumpur, Malaysia, 27 Aug - 30 Aug, 2016.
Presentation Type:
Poster Presentation Session
Topic:
14th Meeting of the Asian-Pacific Society for Neurochemistry
Citation:
Bala
U,
Fauziah
O,
Lai
MI,
Ling
K,
Hayati
KS and
Cheah
P
(2016). Ts1Cje mouse model for Down syndrome exhibits motor function deficit; the potential role of skeletal muscles and peripheral nervous system.
Conference Abstract:
14th Meeting of the Asian-Pacific Society for Neurochemistry.
doi: 10.3389/conf.fncel.2016.36.00181
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Received:
04 Aug 2016;
Published Online:
11 Aug 2016.
*
Correspondence:
Mr. Usman Bala, Universiti Putra Malaysia, NeuroBiology and Genetics Group, Genetics and Regenerative Medicine Research Centre, Faculty of Medicine and Health Sciences, Serdang, Selangor, Malaysia, balausman33@yahoo.com
Dr. Pike-See Cheah, Universiti Putra Malaysia, NeuroBiology and Genetics Group, Genetics and Regenerative Medicine Research Centre, Faculty of Medicine and Health Sciences, Serdang, Selangor, Malaysia, cheahpikesee@upm.edu.my