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DOI: 10.14670/HH-18-017
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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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Fichero | Descripción | Tamaño | Formato | |
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Qiao-33-1311-1321-2018.pdf | 21,04 MB | Adobe PDF | Visualizar/Abrir |
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