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The severity of SMA is directly correlated with SMN protein levels in affected patients; however, the cellular regulatory mechanisms for SMN protein expression are not completely understood. In this study, we investigated the regulatory effects between SMN expression and miR\u20109a, a downstream noncoding small RNA. Using an inducible SMN short hairpin RNA interference (shRNAi) system in NSC 34 and human skin fibroblast cells, cellular miR\u20109 levels and SMN protein repression were time\u2010dependently upregulated. Conversely, cellular miR\u20109 levels decreased when HeLa cells were transfected with SMN protein fused with green fluorescent protein. In SMA\u2010like mice spinal cords and human primary skin fibroblasts isolated from patients with different degrees of SMA, human SMN exhibited a disease severity\u2010dependent decrease, whereas cellular miR\u20109 levels increased. These results clearly suggested that cellular SMN proteins regulated miR\u20109 expression and that miR\u20109 expression was related to SMA severity. Thus, miR\u20109 may be a marker for SMA prognosis.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1016\/j.kjms.2013.12.007","type":"journal-article","created":{"date-parts":[[2014,1,31]],"date-time":"2014-01-31T16:46:57Z","timestamp":1391186817000},"page":"229-234","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":21,"title":["Survival of motor neuron protein downregulates miR\u20109 expression in patients with spinal muscular 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