Por favor, use este identificador para citar o enlazar este ítem: DOI: 10.14670/HH-18-017

Título: Altered myelination in the Niemann-Pick type C1 mutant mouse
Fecha de publicación: 2018
Editorial: Universidad de Murcia. Departamento de Biología Celular e Histología
Cita bibliográfica: Histology and Histopathology, Vol.33, nº12, (2018)
ISSN: 1699-5848
0213-3911
Materias relacionadas: CDU::6 - Ciencias aplicadas::61 - Medicina::616 - Patología. Medicina clínica. Oncología
Palabras clave: NPC1
Myelination
Myelin basic protein
Protein expression
Resumen: Niemann–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.
Autor/es principal/es: Qiao, Liang
Yang, Enhui
Luo, Jiankai
Lin, Juntang
Yan, Xin
URI: http://hdl.handle.net/10201/120813
DOI: DOI: 10.14670/HH-18-017
Tipo de documento: info:eu-repo/semantics/article
Número páginas / Extensión: 11
Derechos: info:eu-repo/semantics/openAccess
Attribution-NonCommercial-NoDerivatives 4.0 Internacional
Aparece en las colecciones:Vol.33,nº12 (2018)

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