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Int J Biol Sci 2016; 12(9):1140-1149. doi:10.7150/ijbs.15938

Research Paper

The Overexpression of TDP-43 Protein in the Neuron and Oligodendrocyte Cells Causes the Progressive Motor Neuron Degeneration in the SOD1 G93A Transgenic Mouse Model of Amyotrophic Lateral Sclerosis

Yi Lu1*, Chunyan Tang1*, Lei Zhu1*, Jiao Li1*, Huiting Liang1, Jie Zhang1, 2, Renshi Xu1✉

1. Department of Neurology, the First Affiliated Hospital of Nanchang University, Nanchang 330006, Jiangxi, China;
2. Department of Biochemistry and Molecular Biology, College of Basic Medical Science, Nanchang University, Nanchang 330006, Jiangxi, China.
* indicates equal contributors.

Abstract

The recent investigation suggested that the TDP-43 protein was closely related to the motor neuron degeneration in amyotrophic lateral sclerosis (ALS), but the pathogenesis contributed to motor neuron degeneration largely remained unknown. Therefore, we detected the alteration of TDP-43 expression and distribution in the adult spinal cord of the SOD1 G93A transgenic mouse model for searching the possible pathogenesis of ALS. We examined the TDP-43 expression and distribution in the different anatomic regions, segments and neural cells in the adult spinal cord at the different stages of the SOD1 wild-type and G93A transgenic model by the fluorescent immunohistochemical technology. We revealed that the amount of TDP-43 positive cell was cervical>lumbar>thoracic segment, that in the ventral horn was more than that in the dorsal horn, a few of TDP-43 protein sparsely expressed and distributed in the other regions, the TDP-43 protein weren't detected in the white matter and the central canal. The TDP-43 protein was mostly expressed and distributed in the nuclear of neuron cells and the cytoplasm of oligodendrocyte cells of the gray matter surrounding the central canal of spinal cord by the granular shape in the SOD1 wild-type and G93A transgenic mice. The amount of TDP-43 positive cell significantly increased at the onset and progression stages of ALS following with the increase of neuron death in spinal cord, particularly in the ventral horn of cervical segment at the progression stage. Our results suggested that the overexpression of TDP-43 protein in the neuron and oligodendrocyte cell causes the progressive motor neuron degeneration in the ALS-like mouse model.

Keywords: Amyotrophic lateral sclerosis. Animal models. Mechanism of neurodegenerative diseases. Motor neuron diseases. Motor neuron. Neurodegeneration. Neurodegenerative disease. SOD1. Transgenic mice.

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How to cite this article:
Lu Y, Tang C, Zhu L, Li J, Liang H, Zhang J, Xu R. The Overexpression of TDP-43 Protein in the Neuron and Oligodendrocyte Cells Causes the Progressive Motor Neuron Degeneration in the SOD1 G93A Transgenic Mouse Model of Amyotrophic Lateral Sclerosis. Int J Biol Sci 2016; 12(9):1140-1149. doi:10.7150/ijbs.15938. Available from http://www.ijbs.com/v12p1140.htm