10.18453/rosdok_id00003713
Yang, Fan
Fan
Yang
http://d-nb.info/gnd/1260687694
Defective myelination and its relative mechanism in Niemann-Pick type C disease
Universität Rostock
2019
500 Natural sciences
570 Life science
610 Medical sciences Medicine
2019
en
http://purl.uni-rostock.de/rosdok/id00003713
urn:nbn:de:gbv:28-rosdok_id00003713-9
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.