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

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 an...

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Autores principales: Rahimi Balaei, Maryam, Jiao, Xiaodan, Ashtari, Niloufar, Afsharinezhad, Pegah, Ghavami, Saeid, Marzban, Hassan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4730356/
https://www.ncbi.nlm.nih.gov/pubmed/26784182
http://dx.doi.org/10.3390/ijms17010115
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author Rahimi Balaei, Maryam
Jiao, Xiaodan
Ashtari, Niloufar
Afsharinezhad, Pegah
Ghavami, Saeid
Marzban, Hassan
author_facet Rahimi Balaei, Maryam
Jiao, Xiaodan
Ashtari, Niloufar
Afsharinezhad, Pegah
Ghavami, Saeid
Marzban, Hassan
author_sort Rahimi Balaei, Maryam
collection PubMed
description 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.
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spelling pubmed-47303562016-02-11 Cerebellar Expression of the Neurotrophin Receptor p75 in Naked-Ataxia Mutant Mouse Rahimi Balaei, Maryam Jiao, Xiaodan Ashtari, Niloufar Afsharinezhad, Pegah Ghavami, Saeid Marzban, Hassan Int J Mol Sci Article 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. MDPI 2016-01-15 /pmc/articles/PMC4730356/ /pubmed/26784182 http://dx.doi.org/10.3390/ijms17010115 Text en © 2016 by the authors; licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons by Attribution (CC-BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Rahimi Balaei, Maryam
Jiao, Xiaodan
Ashtari, Niloufar
Afsharinezhad, Pegah
Ghavami, Saeid
Marzban, Hassan
Cerebellar Expression of the Neurotrophin Receptor p75 in Naked-Ataxia Mutant Mouse
title Cerebellar Expression of the Neurotrophin Receptor p75 in Naked-Ataxia Mutant Mouse
title_full Cerebellar Expression of the Neurotrophin Receptor p75 in Naked-Ataxia Mutant Mouse
title_fullStr Cerebellar Expression of the Neurotrophin Receptor p75 in Naked-Ataxia Mutant Mouse
title_full_unstemmed Cerebellar Expression of the Neurotrophin Receptor p75 in Naked-Ataxia Mutant Mouse
title_short Cerebellar Expression of the Neurotrophin Receptor p75 in Naked-Ataxia Mutant Mouse
title_sort cerebellar expression of the neurotrophin receptor p75 in naked-ataxia mutant mouse
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4730356/
https://www.ncbi.nlm.nih.gov/pubmed/26784182
http://dx.doi.org/10.3390/ijms17010115
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