{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T04:53:53Z","timestamp":1790916833614,"version":"4.1.0"},"reference-count":56,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2024,4,14]],"date-time":"2024-04-14T00:00:00Z","timestamp":1713052800000},"content-version":"vor","delay-in-days":13,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["CNS Neurosci Ther"],"published-print":{"date-parts":[[2024,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Aims<\/jats:title><jats:p>Classification of spinal muscular atrophy (SMA) is associated with the clinical prognosis; however, objective classification markers are scarce. This study aimed to identify metabolic markers in the cerebrospinal fluid (CSF) of children with SMA types II and III.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>CSF samples were collected from 40 patients with SMA (27 with type II and 13 with type III) and analyzed for metabolites.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We identified 135 metabolites associated with SMA types II and III. These were associated with lysine degradation and arginine, proline, and tyrosine metabolism. We identified seven metabolites associated with the Hammersmith Functional Motor Scale: 4\u2010chlorophenylacetic acid, adb\u2010chminaca,(+\/\u2212)\u2010, dodecyl benzenesulfonic acid, norethindrone acetate, 4\u2010(undecan\u20105\u2010yl) benzene\u20101\u2010sulfonic acid, dihydromaleimide beta\u2010d\u2010glucoside, and cinobufagin. Potential typing biomarkers, N\u2010cyclohexylformamide, cinobufagin, cotinine glucuronide, N\u2010myristoyl arginine, 4\u2010chlorophenylacetic acid, geranic acid, 4\u2010(undecan\u20105\u2010yl) benzene, and 7,8\u2010diamino pelargonate, showed good predictive performance. Among these, N\u2010myristoyl arginine was unaffected by the gene phenotype.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This study identified metabolic markers are promising candidate prognostic factors for SMA. We also identified the metabolic pathways associated with the severity of SMA. These assessments can help predict the outcomes of screening SMA classification biomarkers.<\/jats:p><\/jats:sec>","DOI":"10.1111\/cns.14718","type":"journal-article","created":{"date-parts":[[2024,4,14]],"date-time":"2024-04-14T21:06:00Z","timestamp":1713128760000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Metabolomics of cerebrospinal fluid reveals candidate diagnostic biomarkers to distinguish between spinal muscular atrophy type <scp>II<\/scp> and type <scp>III<\/scp>"],"prefix":"10.1111","volume":"30","author":[{"given":"Mengnan","family":"Lu","sequence":"first","affiliation":[{"name":"Department of Pediatrics the Second Affiliated Hospital of Xi'an Jiaotong University  Xi'an Shaanxi China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xueying","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Pediatrics the Second Affiliated Hospital of Xi'an Jiaotong 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