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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
Related subjects: CDU::5 - Ciencias puras y naturales::57 - Biología::576 - Biología celular y subcelular. Citología
Keywords: NPC1
Gene expression
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
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)

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