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Symptomatology goes from severe neuromuscular impairment leading to early death in infants to slow progressing motor deficits during adulthood. Much of the knowledge about the pathophysiology of <jats:styled-content style=\"fixed-case\">SMA<\/jats:styled-content> comes from studies using genetically engineered animal models of the disease. Here we investigated one of the milder models, the homozygous <jats:styled-content style=\"fixed-case\">A<\/jats:styled-content>2<jats:styled-content style=\"fixed-case\">G SMA<\/jats:styled-content> mice, in which the level of the protein is restored to almost normal levels by the addition of a mutated transgene to the severe <jats:styled-content style=\"fixed-case\">SMN<\/jats:styled-content>\u2010deficient background. We examined neuromuscular function and found that calcium\u2010dependent neurotransmitter release was significantly decreased. In addition, the amplitude of spontaneous endplate potentials was decreased, the morphology of <jats:styled-content style=\"fixed-case\">NMJ<\/jats:styled-content> altered, and slight changes in short\u2010term synaptic plasticity were found. In spite of these defects, excitation contraction coupling was well preserved, possibly due to the safety factor of this synapse. These data further support that the quasi\u2010normal restoration of <jats:styled-content style=\"fixed-case\">SMN<\/jats:styled-content> levels in severe cases preserves neuromuscular function, even when neurotransmitter release is significantly decreased at motor nerve terminals. Nevertheless, this deficit could represent a greater risk of motor impairment during aging or after injuries.<\/jats:p>","DOI":"10.1111\/joa.12038","type":"journal-article","created":{"date-parts":[[2013,3,14]],"date-time":"2013-03-14T07:57:17Z","timestamp":1363247837000},"page":"74-84","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["Neurotransmitter release in motor nerve terminals of a mouse model of mild <scp>s<\/scp>pinal <scp>m<\/scp>uscular <scp>a<\/scp>trophy"],"prefix":"10.1111","volume":"224","author":[{"given":"Roc\u00edo","family":"Ruiz","sequence":"first","affiliation":[{"name":"Department of Medical Physiology and Biophysics School of Medicine University of Seville Seville Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luc\u00eda","family":"Tabares","sequence":"additional","affiliation":[{"name":"Department of Medical Physiology and Biophysics School of Medicine University of 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