{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,4]],"date-time":"2026-09-04T11:19:59Z","timestamp":1788520799560,"version":"build-2803163510"},"reference-count":38,"publisher":"SAGE Publications","issue":"5","license":[{"start":{"date-parts":[[2026,1,16]],"date-time":"2026-01-16T00:00:00Z","timestamp":1768521600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,1,16]],"date-time":"2026-01-16T00:00:00Z","timestamp":1768521600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Journal of Neuromuscular Diseases"],"published-print":{"date-parts":[[2026,9]]},"abstract":"<jats:p>\n                    Spinal muscular atrophy (SMA) comprises a spectrum of clinical severities, yet the pathomechanisms of late-onset forms (Type III) remain insufficiently understood. While severe early-onset SMA has been extensively investigated using existing models, their translational relevance to adult disease is limited. Here, we recommend the 4-copy\n                    <jats:italic toggle=\"yes\">SMN2<\/jats:italic>\n                    mouse (FVB.Cg-\n                    <jats:italic toggle=\"yes\">Smn1<\/jats:italic>\n                    tm1Hung Tg(\n                    <jats:italic toggle=\"yes\">SMN2<\/jats:italic>\n                    )2Hung\/J) as the most appropriate model for late-onset SMA. This model exhibits delayed onset, progressive motor dysfunction, and extended survival, enabling the study of chronic neurodegenerative processes, including astrocyte-mediated motor neuron pathology. Its prolonged therapeutic window makes the model suitable for mechanistic and translational investigations of late-onset SMA.\n                  <\/jats:p>","DOI":"10.1177\/22143602251405151","type":"journal-article","created":{"date-parts":[[2026,1,16]],"date-time":"2026-01-16T09:33:42Z","timestamp":1768556022000},"page":"869-874","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":1,"title":["Choosing the optimal mouse model for the study of late-onset spinal muscular atrophy: Why the 4-copy\n                    <i>SMN2<\/i>\n                    model offers ideal translational relevance"],"prefix":"10.1177","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3449-9829","authenticated-orcid":false,"given":"Markus","family":"Leo","sequence":"first","affiliation":[{"name":"Department of Neurology, Center for Translational Neuro- and Behavioural Sciences (C-TNBS), University Medicine Essen, Essen, 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