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Fan Yang

Defective myelination and its relative mechanism in Niemann-Pick type C disease

Universität Rostock, 2019

Abstract: Niemann-Pick type C (NPC) disease is caused by mutations in npc1 or npc2 gene. In this study, we found the maturation of NPC1-/- oligodendrocytes was significantly inhibited, the myelin-related transcription factors Olig1 and Olig2 were downregulated and the nucleocytoplasmic transport of p57Kip2 was inhibited in NPC1-/- oligodendrocytes. Treatment of lovastatin reduced accumulation of cholesterol in the late endosomal and lysosomal, increased the expression of Olig1 and Olig2, recovered the nucleocytoplasmic transport of p57Kip2, and restored the maturation of NPC1-/- oligodendrocytes.

doctoral thesis   free access