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Int. J. Mol. Sci. 2016, 17(1), 115; doi:10.3390/ijms17010115

Cerebellar Expression of the Neurotrophin Receptor p75 in Naked-Ataxia Mutant Mouse

1
Department of Human Anatomy & Cell Science, Faculty of Health Sciences, College of Medicine, University of Manitoba, Winnipeg, MB R3E 0J9, Canada
2
Health Policy Research Center, Shiraz University of Medical Science, Shiraz 713484579, Iran
*
Author to whom correspondence should be addressed.
Academic Editor: Kurt A. Jellinger
Received: 26 October 2015 / Revised: 7 January 2016 / Accepted: 11 January 2016 / Published: 15 January 2016
(This article belongs to the Special Issue Mechanisms of Neurodegeneration)
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Abstract

Spontaneous mutation in the lysosomal acid phosphatase 2 (Acp2) mouse (nax—naked-ataxia mutant mouse) correlates with severe cerebellar defects including ataxia, reduced size and abnormal lobulation as well as Purkinje cell (Pc) degeneration. Loss of Pcs in the nax cerebellum is compartmentalized and harmonized to the classic pattern of gene expression of the cerebellum in the wild type mouse. Usually, degeneration starts in the anterior and posterior zones and continues to the central and nodular zones of cerebellum. Studies have suggested that the p75 neurotrophin receptor (NTR) plays a role in Pc degeneration; thus, in this study, we investigated the p75NTR pattern and protein expression in the cerebellum of the nax mutant mouse. Despite massive Pc degeneration that was observed in the nax mouse cerebellum, p75NTR pattern expression was similar to the HSP25 pattern in nax mice and comparable with wild type sibling cerebellum. In addition, immunoblot analysis of p75NTR protein expression did not show any significant difference between nax and wild type sibling (p > 0.5). In comparison with wild type counterparts, p75NTR pattern expression is aligned with the fundamental cytoarchitecture organization of the cerebellum and is unchanged in the nax mouse cerebellum despite the severe neurodevelopmental disorder accompanied with Pc degeneration. View Full-Text
Keywords: Purkinje cell; p75NTR; gene expression; nax mutant Purkinje cell; p75NTR; gene expression; nax mutant
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Rahimi Balaei, M.; Jiao, X.; Ashtari, N.; Afsharinezhad, P.; Ghavami, S.; Marzban, H. Cerebellar Expression of the Neurotrophin Receptor p75 in Naked-Ataxia Mutant Mouse. Int. J. Mol. Sci. 2016, 17, 115.

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