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Title: | Aberrant expressions of delta-protocadherins in the brain of Npc1 mutant mice |
Issue Date: | 2014 |
Publisher: | F. Hernández y Juan F. Madrid. Universidad de Murcia: Departamento de Biología Celular e Histología |
Citation: | Histology and Histopathology, vol. 29, nº 9, (2014) |
ISSN: | 1699-5848 0213-3911 |
Related subjects: | CDU::5 - Ciencias puras y naturales::57 - Biología::576 - Biología celular y subcelular. Citología |
Keywords: | NPC1 Delta-protocadherins Gene expression Mouse |
Abstract: | Niemann-Pick type C1 (NPC1) disease is an autosomal recessive disorder characterized by dysmyelination and neurodegeneration, which can result in the death of patients in early childhood in some cases. Members of the delta-protocadherins (Pcdhs) play important roles in neurogenesis and brain development. In this study, we compared expression profiles of Pcdhs in the brain between wild-type and Npc1 mutant mice from postnatal day (P) 9 onwards by in situ hybridization. Our data show that laminar distribution of some Pcdhs in the cerebral cortex of Npc1 mutated mice is different from that of wild-type mice. Furthermore, expressions of Pcdhs by oligodendrocytes in the corpus callosum and by Purkinje cells and granular cells in the cerebellum are strongly decreased in Npc1 mutated mice at later stages. Taken together, our data suggest that aberrant expression of Pcdhs is a pathological process accompanied by neurodegeneration in Npc1 mutant mice. |
Primary author: | Yan, Xin Lukas, Jan Lin, Juntang Ernst, Mathias Koczan, Dirk Witt, Martin Fuellen, Georg Wree, Andreas Rolfs, Arndt Luo, Jiankai |
URI: | http://hdl.handle.net/10201/74953 |
Document type: | info:eu-repo/semantics/article |
Number of pages / Extensions: | 15 |
Rights: | info:eu-repo/semantics/openAccess Attribution-NonCommercial-NoDerivatives 4.0 International |
Appears in Collections: | Vol.29, nº 9 (2014) |
Files in This Item:
File | Description | Size | Format | |
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Yan-29-1185-1199-2014.pdf | 47,44 MB | Adobe PDF | View/Open |
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