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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

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dc.contributor.authorKim, Sun-Jung-
dc.contributor.authorLee, Bong-Hee-
dc.contributor.authorLee, Yong Soon-
dc.contributor.authorKang, Kyung-Sun-
dc.date.accessioned2009-08-11T22:38:00Z-
dc.date.available2009-08-11T22:38:00Z-
dc.date.issued2007-07-02-
dc.identifier.citationBiochem. Biophys. Res. Commun. 360 (2007) 593-599en
dc.identifier.issn0006-291X-
dc.identifier.urihttps://hdl.handle.net/10371/6960-
dc.description.abstractNiemann–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.sponsorshipThis 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.isoen-
dc.publisherElsevieren
dc.subjectNiemann–Pick type C diseaseen
dc.subjectNeural stem cellsen
dc.subjectValproic acidsen
dc.subjectA histone deacetylase inhibitoren
dc.titleDefective cholesterol traffic and neuronal differentiation in neural stem cells of Niemann–Pick type C disease improved by valproic acid, a histone deacetylase inhibitoren
dc.typeArticleen
dc.contributor.AlternativeAuthor김선정-
dc.contributor.AlternativeAuthor이봉희-
dc.contributor.AlternativeAuthor이영순-
dc.contributor.AlternativeAuthor강경선-
dc.identifier.doi10.1016/j.bbrc.2007.06.116-
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