{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T00:23:02Z","timestamp":1790986982372,"version":"4.1.0"},"reference-count":57,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2008,7,23]],"date-time":"2008-07-23T00:00:00Z","timestamp":1216771200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Developmental Dynamics"],"published-print":{"date-parts":[[2008,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Spinal muscular atrophy (SMA) is an autosomal recessive disorder that is characterized by the degeneration of the motor neurons of the spinal cord leading to muscle atrophy. SMA is a result of a loss\u2010of\u2010function of the gene <jats:italic>survival motor neuron\u20101<\/jats:italic> (<jats:italic>SMN1<\/jats:italic>). We have chosen to generate a transgenic swine model of SMA for the development and testing of therapeutics and evaluation of toxicology. To this end, we report the first cloning and identification of the swine <jats:italic>SMN1<\/jats:italic> gene and show that there is significant sequence homology between swine and human SMN throughout the coding region. Reverse transcriptase\u2010polymerase chain reaction results demonstrated slight changes in <jats:italic>SMN<\/jats:italic> RNA expression during development and in different tissues. In contrast, protein expression profiles were dramatically different based upon different tissues and developmental stages, consistent with human SMN expression. Porcine SMN localization is consistent with human SMN, localizing diffusely within the cytoplasm and in punctate nuclear structures characteristic of nuclear gems. Importantly, transient transfection of porcine <jats:italic>SMN1<\/jats:italic> in 3813 SMA type 1 fibroblasts demonstrate that porcine <jats:italic>SMN1<\/jats:italic> can rescue the deficiency of SMN protein and gem formation in these cells. These studies provide the first characterization of the porcine <jats:italic>SMN1<\/jats:italic> gene and SMN protein and suggest that a transgenic swine SMA model is feasible. Developmental Dynamics 237:2268\u20132278, 2008. \u00a9 2008 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/dvdy.21642","type":"journal-article","created":{"date-parts":[[2008,7,29]],"date-time":"2008-07-29T02:52:53Z","timestamp":1217299973000},"page":"2268-2278","source":"Crossref","is-referenced-by-count":17,"title":["Identification and characterization of the porcine (<i>Sus scrofa<\/i>) survival motor neuron (<i>SMN1<\/i>) gene: An animal model for therapeutic studies"],"prefix":"10.1002","volume":"237","author":[{"given":"Monique A.","family":"Lorson","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lee D.","family":"Spate","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Randall S.","family":"Prather","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian 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