Altered myelination in the Niemann-Pick type C1 mutant mouse

作者：Qiao, Liang; Yang, Enhui; Luo, Jiankai; Lin, Juntang^*; Yan, Xin^*

来源：Histology and Histopathology, 2018, 33(12): 1311-1321.

DOI：10.14670/HH-18-017

• NPC1
• Myelination
• Myelin basic protein
• Protein expression

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, myclination 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.

• 出版日期2018-12
• 单位新乡医学院