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dc.contributor.authorSorolla Bardají, Maria Alba
dc.contributor.authorRodríguez Colman, Maria José
dc.contributor.authorVall-llaura Espinosa, Núria
dc.contributor.authorVived Maza, Celia
dc.contributor.authorFernández Nogales, Marta
dc.contributor.authorLucas, José J.
dc.contributor.authorFerrer, Isidre
dc.contributor.authorCabiscol Català, Elisa
dc.date.accessioned2016-10-13T17:39:45Z
dc.date.issued2016-06
dc.identifier.issn0885-7490
dc.identifier.urihttp://hdl.handle.net/10459.1/57916
dc.description.abstractOxidative stress has been described as important to Huntington disease (HD) progression. In a previous HD study, we identified several carbonylated proteins, including pyridoxal kinase and antiquitin, both of which are involved in the metabolism of pyridoxal 5A '-phosphate (PLP), the active form of vitamin B6. In the present study, pyridoxal kinase levels were quantified and showed to be decreased both in HD patients and a R6/1 mouse model, compared to control samples. A metabolomic analysis was used to analyze metabolites in brain samples of HD patients and R6/1 mice, compared to control samples using mass spectrometry. This technique allowed detection of increased concentrations of pyridoxal, the substrate of pyridoxal kinase. In addition, PLP, the product of the reaction, was decreased in striatum from R6/1 mice. Furthermore, glutamate and cystathionine, both substrates of PLP-dependent enzymes were increased in HD. This reinforces the hypothesis that PLP synthesis is impaired, and could explain some alterations observed in the disease. Together, these results identify PLP as a potential therapeutic agent.
dc.description.sponsorshipThis work has been supported by grant BFU2010-17387 from the Ministerio de Educación y Ciencia (Spain).
dc.format.mimetypeapplication/pdf
dc.language.isoeng
dc.publisherSpringer Science, Business Media
dc.relationMICINN/PN2008-2011/BFU2010-17387
dc.relation.isformatofReproducció del document publicat a: https://doi.org/10.1007/s11011-015-9777-7
dc.relation.ispartofMetabolic Brain Disease, 2016, vol. 31, num. 3, p. 579-586
dc.rights(c) Springer Science, Business Media, 2015
dc.subject.classificationEstrès oxidatiu
dc.subject.classificationFosfats
dc.subject.otherOxidative stress
dc.subject.otherPhosphates
dc.titleImpaired PLP-dependent metabolism in brain samples from Huntington disease patients and transgenic R6/1 mice
dc.typeinfo:eu-repo/semantics/article
dc.date.updated2016-10-13T17:39:46Z
dc.identifier.idgrec023606
dc.type.versioninfo:eu-repo/semantics/publishedVersion
dc.rights.accessRightsinfo:eu-repo/semantics/restrictedAccess
dc.identifier.doihttps://doi.org/10.1007/s11011-015-9777-7
dc.date.embargoEndDate2025-01-01


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