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Int. J. Mol. Sci. 2017, 18(10), 2173; doi:10.3390/ijms18102173

Nrf2-Inducers Counteract Neurodegeneration in Frataxin-Silenced Motor Neurons: Disclosing New Therapeutic Targets for Friedreich’s Ataxia

1
Unit of Neuromuscular and Neurodegenerative Diseases, IRCCS Bambino Gesù Children’s Hospital, Viale San Paolo 15, 00146 Rome, Italy
2
Drexel University College of Medicine, 2900 Queen Lane, Philadelphia, PA 19129, USA
3
Laboratory of Biochemistry, IRCCS Bambino Gesù Children’s Hospital, Viale San Paolo 15, 00146 Rome, Italy
4
Movement Analysis and Robotics Laboratory (MARLab), Neurorehabilitation Unit, Department of Neurosciences, IRCCS Bambino Gesù Children’s Hospital, Via Torre di Palidoro, Passoscuro Fiumicino, 00050 Rome, Italy
These authors contributed equally to this work.
*
Author to whom correspondence should be addressed.
Received: 12 September 2017 / Revised: 9 October 2017 / Accepted: 14 October 2017 / Published: 18 October 2017
(This article belongs to the Special Issue Nrf2 in Redox Signaling: A Double Edged Sword)
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Abstract

Oxidative stress is actively involved in Friedreich’s Ataxia (FA), thus pharmacological targeting of the antioxidant machinery may have therapeutic value. Here, we analyzed the relevance of the antioxidant phase II response mediated by the transcription factor Nrf2 on frataxin-deficient cultured motor neurons and on fibroblasts of patients. The in vitro treatment of the potent Nrf2 activator sulforaphane increased Nrf2 protein levels and led to the upregulation of phase II antioxidant enzymes. The neuroprotective effects were accompanied by an increase in neurites’ number and extension. Sulforaphane (SFN) is a natural compound of many diets and is now being used in clinical trials for other pathologies. Our results provide morphological and biochemical evidence to endorse a neuroprotective strategy that may have therapeutic relevance for FA. The findings of this work reinforce the crucial importance of Nrf2 in FA and provide a rationale for using Nrf2-inducers as pharmacological agents. View Full-Text
Keywords: oxidative stress; Nrf2; Friedreich’s Ataxia; sulforaphane; dimethyl fumarate; inducers oxidative stress; Nrf2; Friedreich’s Ataxia; sulforaphane; dimethyl fumarate; inducers
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MDPI and ACS Style

Petrillo, S.; Piermarini, E.; Pastore, A.; Vasco, G.; Schirinzi, T.; Carrozzo, R.; Bertini, E.; Piemonte, F. Nrf2-Inducers Counteract Neurodegeneration in Frataxin-Silenced Motor Neurons: Disclosing New Therapeutic Targets for Friedreich’s Ataxia. Int. J. Mol. Sci. 2017, 18, 2173.

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