Liver growth factor (LGF) upregulates frataxin protein expression and reduces oxidative stress in friedreich’s ataxia transgenic mice

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dc.contributor.author Calatrava-Ferreras, Lucía
dc.contributor.author Gonzalo-Gobernado, Rafael
dc.contributor.author Reimers, Diana
dc.contributor.author Herranz, Antonio S.
dc.contributor.author Casarejos, María J.
dc.contributor.author Jiménez-Escrig, Adriano
dc.contributor.author Regadera, Javier
dc.contributor.author Velasco-Martín, Juan Pedro
dc.contributor.author Vallejo-Muñoz, Manuela
dc.contributor.author Díaz-Gil, Juan José
dc.contributor.author Bazán, Eulalia
dc.contributor.other UAM. Departamento de Anatomía, Histología y Neurociencia es_ES
dc.date.accessioned 2017-04-26T13:48:53Z
dc.date.available 2017-04-26T13:48:53Z
dc.date.issued 2016-12-09
dc.identifier.citation International Journal of Molecular Sciences 17.12 (2016): 1-17 en_US
dc.identifier.issn 1661-6596 (print) es_ES
dc.identifier.issn 1422-0067 (online) es_ES
dc.identifier.uri http://hdl.handle.net/10486/678033
dc.description.abstract Friedreich’s ataxia (FA) is a severe disorder with autosomal recessive inheritance that is caused by the abnormal expansion of GAA repeat in intron 1 of FRDA gen. This alteration leads to a partial silencing of frataxin transcription, causing a multisystem disorder disease that includes neurological and non-neurological damage. Recent studies have proven the effectiveness of neurotrophic factors in a number of neurodegenerative diseases. Therefore, we intend to determine if liver growth factor (LGF), which has a demonstrated antioxidant and neuroprotective capability, could be a useful therapy for FA. To investigate the potential therapeutic activity of LGF we used transgenic mice of the FXNtm1MknTg (FXN)YG8Pook strain. In these mice, intraperitoneal administration of LGF (1.6 µg/mouse) exerted a neuroprotective effect on neurons of the lumbar spinal cord and improved cardiac hypertrophy. Both events could be the consequence of the increment in frataxin expression induced by LGF in spinal cord (1.34-fold) and heart (1.2-fold). LGF also upregulated by 2.6-fold mitochondrial chain complex IV expression in spinal cord, while in skeletal muscle it reduced the relation oxidized glutathione/reduced glutathione. Since LGF partially restores motor coordination, we propose LGF as a novel factor that may be useful in the treatment of FA. en_US
dc.description.sponsorship This work was funded by the Pedro Laín Entralgo Agency (NDG7/09) and the Moving Ataxias Foundation. Lucía Calatrava-Ferreras and Rafael Gonzalo-Gobernado were the recipients of a Pedro Laín Entralgo Agency and a Research Supporting Staff Grant Contract [Instituto de Salud Carlos III], respectively en_US
dc.format.extent 17 pag. es_ES
dc.format.mimetype application/pdf en
dc.language.iso eng en
dc.publisher MDPI en_US
dc.relation.ispartof International Journal of Molecular Sciences en_US
dc.rights © 2016 by the authors en_US
dc.subject.other Frataxin en_US
dc.subject.other Friedreich’s ataxia en_US
dc.subject.other Liver growth factor en_US
dc.subject.other Neuroprotection en_US
dc.subject.other Oxidative stress en_US
dc.title Liver growth factor (LGF) upregulates frataxin protein expression and reduces oxidative stress in friedreich’s ataxia transgenic mice en_US
dc.type article en
dc.subject.eciencia Medicina es_ES
dc.relation.publisherversion http://dx.doi.org/10.3390/ijms17122066 es_ES
dc.identifier.doi 10.3390/ijms17122066 es_ES
dc.identifier.publicationfirstpage 1 es_ES
dc.identifier.publicationissue 12 es_ES
dc.identifier.publicationlastpage 17 es_ES
dc.identifier.publicationvolume 17 es_ES
dc.type.version info:eu-repo/semantics/publishedVersion en
dc.rights.cc Reconocimiento es_ES
dc.rights.accessRights openAccess en
dc.authorUAM Regadera González, Javier Fco. (259324)


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