{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,25]],"date-time":"2026-08-25T21:39:34Z","timestamp":1787693974826,"version":"build-2784847793"},"reference-count":62,"publisher":"MDPI AG","issue":"15","license":[{"start":{"date-parts":[[2024,7,31]],"date-time":"2024-07-31T00:00:00Z","timestamp":1722384000000},"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>The most prevalent rare genetic disease affecting young individuals is spinal muscular atrophy (SMA), which is caused by a loss-of-function mutation in the telomeric gene survival motor neuron (SMN) 1. The high heterogeneity of the SMA pathophysiology is determined by the number of copies of SMN2, a separate centromeric gene that can transcribe for the same protein, although it is expressed at a slower rate. SMA affects motor neurons. However, a variety of different tissues and organs may also be affected depending on the severity of the condition. Novel pharmacological treatments, such as Spinraza, Onasemnogene abeparvovec-xioi, and Evrysdi, are considered to be disease modifiers because their use can change the phenotypes of the patients. Since oxidative stress has been reported in SMA-affected cells, we studied the impact of antioxidant therapy on neural stem cells (NSCs) that have the potential to differentiate into motor neurons. Antioxidants can act through various pathways; for example, some of them exert their function through nuclear factor (erythroid-derived 2)-like 2 (NRF2). We found that curcumin is able to induce positive effects in healthy and SMA-affected NSCs by activating the nuclear translocation of NRF2, which may use a different mechanism than canonical redox regulation through the antioxidant-response elements and the production of antioxidant molecules.<\/jats:p>","DOI":"10.3390\/ijms25158364","type":"journal-article","created":{"date-parts":[[2024,7,31]],"date-time":"2024-07-31T08:02:27Z","timestamp":1722412947000},"page":"8364","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Physiological Features of the Neural Stem Cells Obtained from an Animal Model of Spinal Muscular Atrophy and Their Response to Antioxidant Curcumin"],"prefix":"10.3390","volume":"25","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9656-3219","authenticated-orcid":false,"given":"Raffaella","family":"Adami","sequence":"first","affiliation":[{"name":"Section of Pharmacology and Biosciences, Department of Pharmaceutical Sciences, University of Milan, Via Balzaretti 9, 20133 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matteo","family":"Pezzotta","sequence":"additional","affiliation":[{"name":"Section of Pharmacology and Biosciences, Department of Pharmaceutical Sciences, University of Milan, Via Balzaretti 9, 20133 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Cadile","sequence":"additional","affiliation":[{"name":"Human Physiology Unit, Department of Molecular Medicine, University of Pavia, Via Forlanini 6, 27100 Pavia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Beatrice","family":"Cuniolo","sequence":"additional","affiliation":[{"name":"Section of Pharmacology and Biosciences, Department of Pharmaceutical Sciences, University of Milan, Via Balzaretti 9, 20133 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8788-9783","authenticated-orcid":false,"given":"Gianenrico","family":"Rovati","sequence":"additional","affiliation":[{"name":"Section of Pharmacology and Biosciences, Department of Pharmaceutical Sciences, University of Milan, Via Balzaretti 9, 20133 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Monica","family":"Canepari","sequence":"additional","affiliation":[{"name":"Human Physiology Unit, Department of Molecular Medicine, University of Pavia, Via Forlanini 6, 27100 Pavia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7951-9512","authenticated-orcid":false,"given":"Daniele","family":"Bottai","sequence":"additional","affiliation":[{"name":"Section of Pharmacology and Biosciences, Department of Pharmaceutical Sciences, University of Milan, Via Balzaretti 9, 20133 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,7,31]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"276","DOI":"10.1001\/archneur.1971.00490030102014","article-title":"Two early infantile hereditary cases of progressive muscular atrophy simulating dystrophy, but on a neural basis. 1891","volume":"25","author":"Werdnig","year":"1971","journal-title":"Arch. 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