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To gain insight into the pathogenesis of SMA, we have compared synaptic function of motor terminals in wild-type and severe SMA mice at different ages and in two proximal muscles. Our results show that mutant muscle fibers fire normal action potentials and that multi-innervated terminals are functional. By studying the characteristics of the three main components of synaptic transmission in nerve terminals (spontaneous, evoked, and asynchronous release), we found that the kinetics of the postsynaptic potentials are slowed and evoked neurotransmitter release is decreased by \u223c55%. In addition, asynchronous release is increased \u223c300%, indicating an anomalous augmentation of intraterminal bulk Ca<jats:sup>2+<\/jats:sup>during repetitive stimulation. Together, these results show that the reduction of SMN affects synaptic maturation, evoked release, and regulation of intraterminal Ca<jats:sup>2+<\/jats:sup>levels.<\/jats:p>","DOI":"10.1523\/jneurosci.4496-09.2010","type":"journal-article","created":{"date-parts":[[2010,1,20]],"date-time":"2010-01-20T20:23:27Z","timestamp":1264019007000},"page":"849-857","source":"Crossref","is-referenced-by-count":153,"title":["Altered Intracellular Ca<sup>2+<\/sup>Homeostasis in Nerve Terminals of Severe Spinal Muscular Atrophy Mice"],"prefix":"10.1523","volume":"30","author":[{"given":"Roc\u00edo","family":"Ruiz","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juan 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