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Defective cholesterol traffic and neuronal differentiation in neural stem cells of Niemann–Pick type C disease improved by valproic acid, a histone deacetylase inhibitor
DC Field | Value | Language |
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dc.contributor.author | Kim, Sun-Jung | - |
dc.contributor.author | Lee, Bong-Hee | - |
dc.contributor.author | Lee, Yong Soon | - |
dc.contributor.author | Kang, Kyung-Sun | - |
dc.date.accessioned | 2009-08-11T22:38:00Z | - |
dc.date.available | 2009-08-11T22:38:00Z | - |
dc.date.issued | 2007-07-02 | - |
dc.identifier.citation | Biochem. Biophys. Res. Commun. 360 (2007) 593-599 | en |
dc.identifier.issn | 0006-291X | - |
dc.identifier.uri | https://hdl.handle.net/10371/6960 | - |
dc.description.abstract | Niemann–Pick type C disease (NPC) is a neurodegenerative and lipid storage disorder for which no effective treatment is known. We previously reported that neural stem cells derived from NPC1 mice showed impaired self-renewal and differentiation. We examined whether valproic acid (VPA), a histone deacetylase inhibitor, could enhance neuronal differentiation and recover defective cholesterol metabolism in neural stem cells (NSCs) from NPC1-deficient mice (NPC1−/−). VPA could induce neuronal differentiation and restore impaired astrocytes in NSCs from NPC1−/− mice. Importantly, an increasing level of cholesterol within NSCs from NPC1−/− mice could be reduced by VPA. Moreover, essential neurotrophic genes (TrkB, BDNF, MnSoD, and NeuroD) were up-regulated through the repression of the REST/NRSF and HDAC complex by the VPA treatment. Up-regulated neurotrophic genes were able to enhance neural differentiation and cholesterol homeostasis in neural stem cells from NPC1−/− mice. In this study, we suggested that, along with cholesterol homeostasis, impaired neuronal differentiation and abnormal morphology of astrocytes could be rescued by the inhibition of HDAC and REST/NRSF activity induced by VPA treatment. | en |
dc.description.sponsorship | This work was supported by a grant from the Korean Science & Engineering Foundation (R01-2005-000-10190-0), and was further supported by the BK21 Program for Veterinary Science. | en |
dc.language.iso | en | - |
dc.publisher | Elsevier | en |
dc.subject | Niemann–Pick type C disease | en |
dc.subject | Neural stem cells | en |
dc.subject | Valproic acids | en |
dc.subject | A histone deacetylase inhibitor | en |
dc.title | Defective cholesterol traffic and neuronal differentiation in neural stem cells of Niemann–Pick type C disease improved by valproic acid, a histone deacetylase inhibitor | en |
dc.type | Article | en |
dc.contributor.AlternativeAuthor | 김선정 | - |
dc.contributor.AlternativeAuthor | 이봉희 | - |
dc.contributor.AlternativeAuthor | 이영순 | - |
dc.contributor.AlternativeAuthor | 강경선 | - |
dc.identifier.doi | 10.1016/j.bbrc.2007.06.116 | - |
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