Publication:
Altered myelination in the Niemann-Pick type C1 mutant mouse

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dc.contributor.authorQiao, Liang
dc.contributor.authorYang, Enhui
dc.contributor.authorLuo, Jiankai
dc.contributor.authorLin, Juntang
dc.contributor.authorYan, Xin
dc.date.accessioned2022-06-02T11:21:40Z
dc.date.available2022-06-02T11:21:40Z
dc.date.issued2018
dc.description.abstractNiemann–Pick type C1 (NPC1) disease is a lysosomal storage disorder caused by mutation of Npc1 or Npc2 gene, resulting in various progressive pathological features. Myelin defection is a major pathological problem in Npc1 mutant mice; however, impairment of myelin proteins in the developing brain is still incompletely understood. In this study, we showed that the expression of myelin genes and proteins is strongly inhibited from postnatal day 35 onwards including reduced myelin basic protein (MBP) expression in the brain. Furthermore, myelination characterized by MBP immunohistochemistry was strongly perturbed in the forebrain, moderately in the midbrain and cerebellum, and slightly in the hindbrain. Our results demonstrate that mutation of the Npc1 gene is sufficient to cause severe and progressive defects in myelination in the mouse brain.es
dc.formatapplication/pdfes
dc.format.extent11es
dc.identifier.doiDOI: 10.14670/HH-18-017
dc.identifier.eisbnHistology and Histopathology, Vol.33, nº12, (2018)es
dc.identifier.issn1699-5848
dc.identifier.issn0213-3911
dc.identifier.urihttp://hdl.handle.net/10201/120813
dc.languageenges
dc.publisherUniversidad de Murcia. Departamento de Biología Celular e Histologíaes
dc.relationSin financiación externa a la Universidades
dc.rightsinfo:eu-repo/semantics/openAccesses
dc.rightsAttribution-NonCommercial-NoDerivatives 4.0 Internacional*
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/*
dc.subjectNPC1es
dc.subjectMyelinationes
dc.subjectMyelin basic proteines
dc.subjectProtein expressiones
dc.subject.otherCDU::6 - Ciencias aplicadas::61 - Medicina::616 - Patología. Medicina clínica. Oncologíaes
dc.titleAltered myelination in the Niemann-Pick type C1 mutant mousees
dc.typeinfo:eu-repo/semantics/articlees
dspace.entity.typePublicationes
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Histology and histopathology Vol.33,nº12 (2018)