{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,23]],"date-time":"2026-01-23T20:18:04Z","timestamp":1769199484887,"version":"3.49.0"},"reference-count":32,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2014,4,16]],"date-time":"2014-04-16T00:00:00Z","timestamp":1397606400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2014,5]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:p><jats:italic>Introduction<\/jats:italic>: The purpose of this study was to measure and analyze motor unit number estimation (MUNE) values longitudinally in spinal muscular atrophy (SMA). <jats:italic>Methods<\/jats:italic>: Sixty\u2010two children with SMA types 2 and 3 were observed prospectively for up to 42 months. Longitudinal electrophysiological data were collected, including compound motor action potential (CMAP), single motor unit action potential (SMUP), and MUNE. <jats:italic>Results<\/jats:italic>: Significant motor neuron loss and compensatory collateral reinnervation were noted at baseline. Over time, there was a significant mean increase in MUNE (4.92 units\/year, <jats:italic>P<\/jats:italic>\u2009=\u20090.009), a mean decrease in SMUP amplitude (\u22126.32 \u03bcV\/year, <jats:italic>P<\/jats:italic>\u2009=\u20090.10), and stable CMAP amplitude. <jats:italic>Conclusions<\/jats:italic>: The unexpected longitudinal results differ from findings in amyotrophic lateral sclerosis studies, perhaps indicating that compensatory processes in SMA involve new motor unit development. A better understanding of the mechanisms of motor unit decline and compensation in SMA is important for assessing novel therapeutic strategies and for providing key insights into disease pathophysiology. <jats:italic>Muscle Nerve<\/jats:italic> <jats:bold>49<\/jats:bold>: 636\u2013644, 2014<\/jats:p>","DOI":"10.1002\/mus.23967","type":"journal-article","created":{"date-parts":[[2013,7,24]],"date-time":"2013-07-24T21:16:12Z","timestamp":1374700572000},"page":"636-644","source":"Crossref","is-referenced-by-count":40,"title":["The motor neuron response to <i>SMN1<\/i> deficiency in spinal muscular atrophy"],"prefix":"10.1002","volume":"49","author":[{"given":"Peter B.","family":"Kang","sequence":"first","affiliation":[{"name":"Department of Neurology Boston Children's Hospital and Harvard Medical School 300 Longwood Avenue Boston Massachusetts 02115 USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Clifton 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