{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,4]],"date-time":"2026-08-04T01:01:38Z","timestamp":1785805298780,"version":"3.56.0"},"reference-count":23,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2021,3,13]],"date-time":"2021-03-13T00:00:00Z","timestamp":1615593600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2021,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Nusinersen was approved as the first treatment for all types of spinal muscular atrophy (SMA), including adults with SMA types 2 and 3. Robust biomarkers of treatment response in SMA adults are lacking. Our aim was to examine cerebrospinal fluid (CSF) amyloid\u2010\u03b240 (A\u03b240) and amyloid\u2010\u03b242 (A\u03b242) peptides as biomarkers of treatment response.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Eight patients with SMA types 2 and 3 were recruited consecutively in a single\u2010center study. CSF was sampled at baseline, after a loading dose, and after three maintenance doses. Levels of A\u03b242 and A\u03b240 were evaluated for each CSF sampling. Wilcoxon matched\u2010pairs signed\u2010rank test was used to detect longitudinal changes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>CSF levels of A\u03b242 increased from baseline to day 420 (95% confidence interval, <jats:italic>P<\/jats:italic>\u00a0=\u00a0.018), with a significant increase at days 180 and 420 compared with days 0 and 300, respectively (95% confidence interval, <jats:italic>P<\/jats:italic>\u00a0=\u00a0.012 and <jats:italic>P<\/jats:italic>\u00a0=\u00a0.018).<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>The maintenance and promotion of wellness of residual motor neurons mediated by the restored level of SMN protein due to nusinersen could result in an increased level of amyloid peptides.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.27212","type":"journal-article","created":{"date-parts":[[2021,3,4]],"date-time":"2021-03-04T12:09:35Z","timestamp":1614859775000},"page":"905-909","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["Is cerebrospinal fluid amyloid\u2010\u03b242 a promising biomarker of response to nusinersen in adult spinal muscular atrophy patients?"],"prefix":"10.1002","volume":"63","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3141-6442","authenticated-orcid":false,"given":"Alessandro","family":"Introna","sequence":"first","affiliation":[{"name":"Neurology Unit, Department of Basic Medical Sciences, Neurosciences and Sense Organs University of Bari \"Aldo Moro,\"  Bari Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giammarco","family":"Milella","sequence":"additional","affiliation":[{"name":"Neurology Unit, Department of Basic Medical Sciences, Neurosciences and Sense Organs University of Bari \"Aldo Moro,\"  Bari Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eustachio","family":"D'Errico","sequence":"additional","affiliation":[{"name":"Neurology Unit, Department of Basic Medical Sciences, Neurosciences and Sense Organs University of Bari \"Aldo Moro,\"  Bari Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angela","family":"Fraddosio","sequence":"additional","affiliation":[{"name":"Neurology Unit, Department of Basic Medical Sciences, Neurosciences and Sense Organs University of Bari \"Aldo Moro,\"  Bari Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gaspare","family":"Scaglione","sequence":"additional","affiliation":[{"name":"Neurology Unit, Department of Basic Medical Sciences, Neurosciences and Sense Organs University of Bari \"Aldo Moro,\"  Bari Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Ucci","sequence":"additional","affiliation":[{"name":"Neurology Unit, Department of Basic Medical Sciences, Neurosciences and Sense Organs University of Bari \"Aldo Moro,\"  Bari Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7419-570X","authenticated-orcid":false,"given":"Maddalena","family":"Ruggieri","sequence":"additional","affiliation":[{"name":"Neurophysiopathology Unit, Department of Basic Medical Sciences, Neurosciences and Sense Organs University of Bari \"Aldo Moro,\"  Bari Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7429-3091","authenticated-orcid":false,"given":"Isabella Laura","family":"Simone","sequence":"additional","affiliation":[{"name":"Neurology Unit, Department of Basic Medical Sciences, Neurosciences and Sense Organs University of Bari \"Aldo Moro,\"  Bari Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,3,13]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pcl.2015.03.010"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1710504"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2018-320033"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-019-09389-8"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/jcmm.14939"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.3233\/JND-190416"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijms20215397"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1177\/1756286419846058"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1177\/0883073811420294"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.25430"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.3233\/JND-200534"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijms21082911"},{"key":"e_1_2_11_14_1","doi-asserted-by":"publisher","DOI":"10.1159\/000048642"},{"key":"e_1_2_11_15_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0023600"},{"key":"e_1_2_11_16_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40265-017-0711-7"},{"key":"e_1_2_11_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2020.116756"},{"key":"e_1_2_11_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/jnr.10667"},{"key":"e_1_2_11_19_1","doi-asserted-by":"publisher","DOI":"10.1159\/000065696"},{"key":"e_1_2_11_20_1","unstructured":"ResearchGate. \u03b2\u2010amyloid helps to protect neurons from oxidative stress.https:\/\/www.researchgate.net\/publication\/247225659_b\u2010amyloid_helps_to_protect_neurons_from_oxidative_stress. Accessed September 18 2020."},{"key":"e_1_2_11_21_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.2692-08.2008"},{"key":"e_1_2_11_22_1","doi-asserted-by":"publisher","DOI":"10.1358\/dot.2017.53.6.2652413"},{"key":"e_1_2_11_23_1","doi-asserted-by":"publisher","DOI":"10.1093\/emboj\/20.19.5443"},{"key":"e_1_2_11_24_1","doi-asserted-by":"publisher","DOI":"10.1155\/2014\/795375"}],"container-title":["Muscle &amp; Nerve"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.27212","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/mus.27212","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.27212","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,30]],"date-time":"2023-08-30T00:29:28Z","timestamp":1693355368000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/mus.27212"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,3,13]]},"references-count":23,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2021,6]]}},"alternative-id":["10.1002\/mus.27212"],"URL":"https:\/\/doi.org\/10.1002\/mus.27212","archive":["Portico"],"relation":{},"ISSN":["0148-639X","1097-4598"],"issn-type":[{"value":"0148-639X","type":"print"},{"value":"1097-4598","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,3,13]]},"assertion":[{"value":"2020-09-25","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-02-27","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-03-13","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}