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Aberrant expressions of delta-protocadherins in the brain of Npc1 mutant mice

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dc.contributor.authorYan, Xin
dc.contributor.authorLukas, Jan
dc.contributor.authorLin, Juntang
dc.contributor.authorErnst, Mathias
dc.contributor.authorKoczan, Dirk
dc.contributor.authorWitt, Martin
dc.contributor.authorFuellen, Georg
dc.contributor.authorWree, Andreas
dc.contributor.authorRolfs, Arndt
dc.contributor.authorLuo, Jiankai
dc.date.accessioned2019-09-27T15:20:11Z
dc.date.available2019-09-27T15:20:11Z
dc.date.issued2014
dc.description.abstractNiemann-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.es
dc.formatapplication/pdfes
dc.format.extent15es
dc.identifier.citationHistology and Histopathology, vol. 29, nº 9, (2014)
dc.identifier.issn1699-5848
dc.identifier.issn0213-3911
dc.identifier.urihttp://hdl.handle.net/10201/74953
dc.languageenges
dc.publisherF. Hernández y Juan F. Madrid. Universidad de Murcia: Departamento de Biología Celular e Histologíaes
dc.rightsinfo:eu-repo/semantics/openAccesses
dc.rightsAttribution-NonCommercial-NoDerivatives 4.0 International*
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/*
dc.subjectNPC1es
dc.subjectDelta-protocadherinses
dc.subjectGene expressiones
dc.subjectMousees
dc.subject.otherCDU::5 - Ciencias puras y naturales::57 - Biología::576 - Biología celular y subcelular. Citologíaes
dc.titleAberrant expressions of delta-protocadherins in the brain of Npc1 mutant micees
dc.typeinfo:eu-repo/semantics/articlees
dspace.entity.typePublicationes
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Histology and histopathology Vol.29, nº 9 (2014)