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dc.contributor.author박혜영*
dc.contributor.author정성철*
dc.contributor.author박주원*
dc.date.accessioned2016-08-28T12:08:11Z-
dc.date.available2016-08-28T12:08:11Z-
dc.date.issued2009*
dc.identifier.issn0009-8981*
dc.identifier.otherOAK-5434*
dc.identifier.urihttps://dspace.ewha.ac.kr/handle/2015.oak/220141-
dc.description.abstractBackground: Phenylketonuria (PKU) is an autosomal recessive disorder caused by a deficiency of phenylalanine hydroxylase (PAH), which catalyzes the conversion of phenylalanine to tyrosine. The resultant hyperphenylalaninemia causes mental retardation, seizure, and abnormalities in behavior and movement. Methods: We analyzed gene expression profiles in brain tissues of Pahenu2 mice to elucidate the mechanisms involved in phenylalanine-induced neurological damage. The altered gene expression was confirmed by real-time PCR and Western blotting. To identify markers associated with neurological damage, we examined TTR expression in serum by Western blotting. Results: Gene expression profiling of brain tissue from a mouse model of PKU revealed overexpression of transthyretin (Ttr), sclerostin domain containing 1 (Sostdc1), α-Klotho (Kl), prolactin receptor (Prlr), and early growth response 2 (Egr2). In contrast to its overexpression in the brain, TTR expression was low in the sera of PKU mice offered unrestricted access to a diet containing phenylalanine. Expression of TTR decreased in a time-dependent manner in phenylalanine-treated HepG2 cells. Conclusions: These findings indicate that Ttr, Sostdc1, Kl, Prlr, and Egr2 can be involved in the pathogenesis of PKU and that phenylalanine might have a direct effect on the level of TTR in serum. © 2008 Elsevier B.V. All rights reserved.*
dc.languageEnglish*
dc.titleAltered brain gene expression profiles associated with the pathogenesis of phenylketonuria in a mouse model*
dc.typeArticle*
dc.relation.issue41276*
dc.relation.volume401*
dc.relation.indexSCI*
dc.relation.indexSCIE*
dc.relation.indexSCOPUS*
dc.relation.startpage90*
dc.relation.lastpage99*
dc.relation.journaltitleClinica Chimica Acta*
dc.identifier.doi10.1016/j.cca.2008.11.019*
dc.identifier.wosidWOS:000263797000019*
dc.identifier.scopusid2-s2.0-58549088673*
dc.author.googlePark J.-W.*
dc.author.googlePark E.-S.*
dc.author.googleChoi E.N.*
dc.author.googlePark H.-Y.*
dc.author.googleJung S.-C.*
dc.contributor.scopusid박혜영(7601567979)*
dc.contributor.scopusid정성철(57008539100)*
dc.contributor.scopusid박주원(8656832200)*
dc.date.modifydate20240301081003*
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의과대학 > 의학과 > Journal papers
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