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Expression and Distribution of Arylsulfatase B are Closely Associated with Neuron Death in SOD1 G93A Transgenic Mice

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Abstract

The known proteins only explained the partial pathogenesis of amyotrophic lateral sclerosis (ALS). Therefore, this study aimed to search the novel proteins possibly involved in ALS. In this study, we analyzed the expression and distribution of the candidate protein arylsulfatase B (ARSB) in the different segments, anatomic regions, and neural cells of spinal cord at the different stages of the wild-type and [Cu/Zn] superoxide dismutase 1 (SOD1) G93A transgenic mice using the fluorescent immunohistochemistry and the western blot. The results revealed that the ARSB was extensively expressed and distributed in the entire spinal cord; the expression and distribution of ARSB was significantly different in the different regions of spinal cord, the anterior horn of gray matter (AHGM) was significantly more than that in the posterior horn of gray matter (PHGM) and significantly more than that in the central canal, and ARSB was mainly distributed in the microglia and neuron cells in the wild-type mice. The expression of ARSB significantly increased in other anatomic regions besides the thoracic PHGM, significantly decreased at the progression stage, occurred in the redistribution from the AHGM and the PHGM to the central canal at the onset and progression stages, and no any alteration of ARSB expression and distribution occurred between the different neural cells in the SOD1 G93A mice compared with the wild-type mice. The increase of ARSB expression and distribution followed with the increased of neuron death. Our data suggested that the abnormal expression and distribution of ARSB were closely associated with the neuron death in the SOD1 G93A transgenic mice.

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Abbreviations

ALS:

Amyotrophic lateral sclerosis

ARSB:

Arylsulfatase B

SOD1:

[Cu/Zn] superoxide dismutase 1

GFAP:

Glial fibrillary acidic protein

NeuN:

Neuronal nuclear antigen

Fox3:

RNA binding protein fox-1 homolog 3

IBA-1:

Ionized calcium binding adapter molecule-1

DAPI:

4′,6-diamidino-2-phenylindole

Hm:

Human

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Acknowledgements

We are grateful to the National Natural Science Foundation of China (30560042, 81160161, 81360198); Education Department of Jiangxi Province (GJJ13198); and Jiangxi provincial department of science and technology ([2014]-47, 20142BBG70062) for supporting this work.

Author Contributions

R. X. and J. Z. conceived and designed the experiments and wrote the manuscript. J. Z., H. L., L. Z., W. G., C. T., and J. L. conducted the experiments and analyzed the data. X. Z. contributed some materials. J. Z., H. L., L. Z., W. G., and C. T. are the first authors and they contributed equally to the work.

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Authors and Affiliations

  1. Department of Neurology, The First Affiliated Hospital of Nanchang University, Nanchang, Jiangxi, 330006, China

    Jie Zhang, Huiting Liang, Lei Zhu, Weiming Gan, Chunyan Tang, Jiao Li & Renshi Xu

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  1. Jie Zhang
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  2. Huiting Liang
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  3. Lei Zhu
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Correspondence to Renshi Xu.

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The work was funded by the National Natural Science Foundation of China (30560042, 81160161, 81360198); Education Department of Jiangxi Province (GJJ13198); and Jiangxi Provincial Department of Science and Technology ([2014]-47, 20142BBG70062).

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Jie Zhang, Huiting Liang, and Lei Zhu contributed equally to this work.

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Zhang, J., Liang, H., Zhu, L. et al. Expression and Distribution of Arylsulfatase B are Closely Associated with Neuron Death in SOD1 G93A Transgenic Mice. Mol Neurobiol 55, 1323–1337 (2018). https://doi.org/10.1007/s12035-017-0406-9

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