{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T00:22:30Z","timestamp":1790986950891,"version":"4.1.0"},"reference-count":43,"publisher":"National Academy of Sciences","issue":"24","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2000,11,21]]},"abstract":"<jats:p>\n                    Spinal muscular atrophy (SMA) is attributed to mutations in the\n                    <jats:italic>SMN1<\/jats:italic>\n                    gene, leading to loss of spinal cord motor neurons.\n The neurotropic Sindbis virus vector system was used to investigate a\n role for the survival motor neuron (SMN) protein in regulating neuronal\n apoptosis. Here we show that SMN protects primary neurons and\n differentiated neuron-like stem cells, but not cultured cell lines from\n virus-induced apoptotic death. SMN also protects neurons\n                    <jats:italic>in vivo<\/jats:italic>\n                    and increases survival of virus-infected mice.\n SMN mutants (SMN\u03947 and SMN-Y272C) found in patients with SMA not only\n lack antiapoptotic activity but also are potently\n proapoptotic, causing increased neuronal apoptosis and\n animal mortality. Full-length SMN is proteolytically processed in\n brains undergoing apoptosis or after ischemic injury. Mutation\n of an Asp-252 of SMN abolished cleavage of SMN and increased the\n antiapoptotic function of full-length SMN in neurons. Taken\n together, deletions or mutations of the C terminus of SMN that result\n from proteolysis, splicing (SMN\u03947), or germ-line mutations (e.g.,\n Y272C), produce a proapoptotic form of SMN that may contribute\n to neuronal death in SMA and perhaps other neurodegenerative disorders.\n                  <\/jats:p>","DOI":"10.1073\/pnas.230364197","type":"journal-article","created":{"date-parts":[[2002,7,26]],"date-time":"2002-07-26T10:40:52Z","timestamp":1027680052000},"page":"13312-13317","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":85,"title":["Survival motor neuron protein modulates neuron-specific apoptosis"],"prefix":"10.1073","volume":"97","author":[{"given":"Douglas A.","family":"Kerr","sequence":"first","affiliation":[{"name":"Departments of Neurology, Molecular Microbiology and\r Immunology, Anesthesiology and Critical Care Medicine,\r and Pharmacology and Molecular Sciences, The Johns\r Hopkins Schools of Medicine and Public Health, Baltimore, MD 21205"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jocelyn P.","family":"Nery","sequence":"additional","affiliation":[{"name":"Departments of Neurology, Molecular Microbiology and\r Immunology, Anesthesiology and Critical Care Medicine,\r and Pharmacology and Molecular Sciences, The Johns\r Hopkins Schools of Medicine and Public Health, Baltimore, MD 21205"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard J.","family":"Traystman","sequence":"additional","affiliation":[{"name":"Departments of Neurology, Molecular Microbiology and\r Immunology, Anesthesiology and Critical Care Medicine,\r and Pharmacology and Molecular Sciences, The Johns\r Hopkins Schools of Medicine and Public Health, Baltimore, MD 21205"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B. Nelson","family":"Chau","sequence":"additional","affiliation":[{"name":"Departments of Neurology, Molecular Microbiology and\r Immunology, Anesthesiology and Critical Care Medicine,\r and Pharmacology and Molecular Sciences, The Johns\r Hopkins Schools of Medicine and Public Health, Baltimore, MD 21205"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. 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