Liver growth factor (LGF) upregulates frataxin protein expression and reduces oxidative stress in friedreich’s ataxia transgenic mice
Entidad
UAM. Departamento de Anatomía, Histología y NeurocienciaEditor
MDPIFecha de edición
2016-12-09Cita
10.3390/ijms17122066
International Journal of Molecular Sciences 17.12 (2016): 1-17
ISSN
1661-6596 (print); 1422-0067 (online)DOI
10.3390/ijms17122066Financiado por
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], respectivelyVersión del editor
http://dx.doi.org/10.3390/ijms17122066Materias
Frataxin; Friedreich’s ataxia; Liver growth factor; Neuroprotection; Oxidative stress; MedicinaDerechos
© 2016 by the authorsResumen
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.
Lista de ficheros
Google Scholar:Calatrava-Ferreras, Lucía
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Gonzalo-Gobernado, Rafael
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Reimers, Diana
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Herranz, Antonio S.
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Casarejos, María J.
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Jiménez-Escrig, Adriano
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Regadera, Javier
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Velasco-Martín, Juan Pedro
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Vallejo-Muñoz, Manuela
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Díaz-Gil, Juan José
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Bazán, Eulalia
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