{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T06:53:19Z","timestamp":1790837599845,"version":"4.1.0"},"reference-count":54,"publisher":"MDPI AG","issue":"10","license":[{"start":{"date-parts":[[2017,10,18]],"date-time":"2017-10-18T00:00:00Z","timestamp":1508284800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Oxidative stress is actively involved in Friedreich\u2019s 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\u2019 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.<\/jats:p>","DOI":"10.3390\/ijms18102173","type":"journal-article","created":{"date-parts":[[2017,10,18]],"date-time":"2017-10-18T11:10:00Z","timestamp":1508325000000},"page":"2173","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":72,"title":["Nrf2-Inducers Counteract Neurodegeneration in Frataxin-Silenced Motor Neurons: Disclosing New Therapeutic Targets for Friedreich\u2019s Ataxia"],"prefix":"10.3390","volume":"18","author":[{"given":"Sara","family":"Petrillo","sequence":"first","affiliation":[{"name":"Unit of Neuromuscular and Neurodegenerative Diseases, IRCCS Bambino Ges\u00f9 Children\u2019s Hospital, Viale San Paolo 15, 00146 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emanuela","family":"Piermarini","sequence":"additional","affiliation":[{"name":"Unit of Neuromuscular and Neurodegenerative Diseases, IRCCS Bambino Ges\u00f9 Children\u2019s Hospital, Viale San Paolo 15, 00146 Rome, Italy"},{"name":"Drexel University College of Medicine, 2900 Queen Lane, Philadelphia, PA 19129, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0990-9419","authenticated-orcid":false,"given":"Anna","family":"Pastore","sequence":"additional","affiliation":[{"name":"Laboratory of Biochemistry, IRCCS Bambino Ges\u00f9 Children\u2019s Hospital, Viale San Paolo 15, 00146 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gessica","family":"Vasco","sequence":"additional","affiliation":[{"name":"Movement Analysis and Robotics Laboratory (MARLab), Neurorehabilitation Unit, Department of Neurosciences, IRCCS Bambino Ges\u00f9 Children\u2019s Hospital, Via Torre di Palidoro, Passoscuro Fiumicino, 00050 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tommaso","family":"Schirinzi","sequence":"additional","affiliation":[{"name":"Movement Analysis and Robotics Laboratory (MARLab), Neurorehabilitation Unit, Department of Neurosciences, IRCCS Bambino Ges\u00f9 Children\u2019s Hospital, Via Torre di Palidoro, Passoscuro Fiumicino, 00050 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rosalba","family":"Carrozzo","sequence":"additional","affiliation":[{"name":"Unit of Neuromuscular and Neurodegenerative Diseases, IRCCS Bambino Ges\u00f9 Children\u2019s Hospital, Viale San Paolo 15, 00146 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9276-4590","authenticated-orcid":false,"given":"Enrico","family":"Bertini","sequence":"additional","affiliation":[{"name":"Unit of Neuromuscular and Neurodegenerative Diseases, IRCCS Bambino Ges\u00f9 Children\u2019s Hospital, Viale San Paolo 15, 00146 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1825-9347","authenticated-orcid":false,"given":"Fiorella","family":"Piemonte","sequence":"additional","affiliation":[{"name":"Unit of Neuromuscular and Neurodegenerative Diseases, IRCCS Bambino Ges\u00f9 Children\u2019s Hospital, Viale San Paolo 15, 00146 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2017,10,18]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1016\/j.freeradbiomed.2015.05.039","article-title":"Oxidative stress in inherited mitochondrial diseases","volume":"88","author":"Hayashi","year":"2015","journal-title":"Free Radic. 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