Defective myelination and its relative mechanism in Niemann-Pick type C disease Yang, Fan , 1986- (VerfasserIn) Luo, Jiankai , 1963- (AkademischeR BetreuerIn) Köhling, Rüdiger , 1966- (AkademischeR BetreuerIn) Pufe, Thomas , 1972- (AkademischeR BetreuerIn) Universität Rostock (Grad-verleihende Institution) Universitätsmedizin Rostock (Grad-verleihende Institution) Text theses Text Hochschulschrift 2019 2019 eng electronic resourceremoteComputermedienOnline-Ressource1 Online-Ressource 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.<eng> vorgelegt von Fan Yang GutachterInnen: Jiankai Luo (Universitätsmedizin Rostock) ; Rüdiger Köhling (Universitätsmedizin Rostock) ; Thomas Pufe (Universitätsklinikum Aachen) Dissertation Universität Rostock 2022 500 570 610 616.8 610 YC 7417 44.37 44.77 http://purl.uni-rostock.de/rosdok/id00003713 https://doi.org/10.18453/rosdok_id00003713 https://nbn-resolving.org/urn:nbn:de:gbv:28-rosdok_id00003713-9 https://d-nb.info/1293537683/34 Defective myelination and its relative mechanism in Niemann-Pick type C disease--(DE-627)1808047729 urn:nbn:de:gbv:28-rosdok_id00003713-9 doi: 10.18453/rosdok_id00003713 oclc: 1331713566 ppn: (DE-627)1807555275