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Impaired functions of neural stem cells by abnormal nitric oxide-mediated signaling in an in vitro model of Niemann-Pick type C disease

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dc.contributor.authorKim, Sun-Jung-
dc.contributor.authorLim, Myung-Sin-
dc.contributor.authorKang, Soo Kyung-
dc.contributor.authorLee, Yong Soon-
dc.contributor.authorKang, Kyung-Sun-
dc.date.accessioned2009-08-26T22:41:49Z-
dc.date.available2009-08-26T22:41:49Z-
dc.date.issued2008-04-08-
dc.identifier.citationCell Res 2008; 18:686-694en
dc.identifier.issn1001-0602-
dc.identifier.urihttps://hdl.handle.net/10371/7890-
dc.description.abstractNitric oxide (NO) has been implicated in the promotion of neurodegeneration. However, little is known about the relationship between NO and the self-renewal or differentiation capacity of neural stem cells (NSCs) in neurodegenerative disease. In this study, we investigated the effect of NO on self-renewal of NSCs in an animal model for Niemann-Pick type C (NPC) disease. We found that NO production was significantly increased in NSCs from NPC1-deficient mice (NPC1- /- ), which showed reduced NSC self-renewal. The number of nestin-positive cells and the size of neurospheres were both significantly decreased. The expression of NO synthase (NOS) was increased in neurospheres derived from the brain of NPC1- /- mice in comparison to wild-type neurospheres. NO-mediated activation of glycogen synthase kinase-3 (GSK3) and caspase-3 was also observed in NSCs from NPC1- /- mice. The self-renewal ability of NSCs from NPC1- /- mice was restored by an NOS inhibitor, L-NAME, which resulted in the inhibition of GSK3 and caspase-3. In addition, the differentiation ability of NSCs was partially restored and the number of Fluoro-Jade C-positive degenerating neurons was reduced. These data suggest that overproduction of NO in NPC disease impaired the self-renewal of NSCs. Control of NO production may be key for the treatment of NPC disease.en
dc.description.sponsorshipThis work was supported by a grant from the Korean
Science & Engineering Foundation (R01-2005-000-10190-0)
and the BK21 Program for Veterinary Science.
en
dc.language.isoenen
dc.publisherNature Publishing Groupen
dc.subjectneural stem cellsen
dc.subjectnitric oxideen
dc.subjectGSK3βen
dc.subjectNiemann-Pick type C1 diseaseen
dc.subjectcaspase-3en
dc.titleImpaired functions of neural stem cells by abnormal nitric oxide-mediated signaling in an in vitro model of Niemann-Pick type C diseaseen
dc.typeArticleen
dc.contributor.AlternativeAuthor김선중-
dc.contributor.AlternativeAuthor임명신-
dc.contributor.AlternativeAuthor강수경-
dc.contributor.AlternativeAuthor이영순-
dc.contributor.AlternativeAuthor강경선-
dc.identifier.doi10.1038/cr.2008.48-
Appears in Collections:
College of Veterinary Medicine (수의과대학)Dept. of Veterinary Medicine (수의학과)Journal Papers (저널논문_수의학과)
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