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Int. J. Mol. Sci. 2016, 17(12), 2066;

Liver Growth Factor (LGF) Upregulates Frataxin Protein Expression and Reduces Oxidative Stress in Friedreich’s Ataxia Transgenic Mice

Service of Neurobiology, Ramón y Cajal Institute for Health Research (IRYCIS), 28034 Madrid, Spain
Service of Neurology, Ramón y Cajal Hospital, 28034 Madrid, Spain
Departamento de Anatomía, Histología y Neurociencia Facultad de Medicina Universidad Autónoma de Madrid, 28400 Madrid, Spain
These authors contributed equally to this work.
Deceased on 4 November 2016.
Author to whom correspondence should be addressed.
Academic Editor: Katalin Prokai-Tatrai
Received: 31 August 2016 / Revised: 28 November 2016 / Accepted: 6 December 2016 / Published: 9 December 2016
(This article belongs to the Special Issue Neuroprotective Strategies 2016)
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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. View Full-Text
Keywords: liver growth factor; Friedreich’s ataxia; neuroprotection; oxidative stress; frataxin liver growth factor; Friedreich’s ataxia; neuroprotection; oxidative stress; frataxin

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Calatrava-Ferreras, L.; Gonzalo-Gobernado, R.; Reimers, D.; Herranz, A.S.; Casarejos, M.J.; Jiménez-Escrig, A.; Regadera, J.; Velasco-Martín, J.; Vallejo-Muñoz, M.; Díaz-Gil, J.J.; Bazán, E. Liver Growth Factor (LGF) Upregulates Frataxin Protein Expression and Reduces Oxidative Stress in Friedreich’s Ataxia Transgenic Mice. Int. J. Mol. Sci. 2016, 17, 2066.

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