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Dystrophin is present intracellularly at the sarcolemma, connecting actin to the dystrophin\u2010associated glycoprotein complex. Interestingly, it is enriched postsynaptically at the neuromuscular junction (\n                    <jats:styled-content style=\"fixed-case\">NMJ<\/jats:styled-content>\n                    ), but its synaptic function is largely unknown. Utrophin, a dystrophin homologue, is also concentrated at the\n                    <jats:styled-content style=\"fixed-case\">NMJ<\/jats:styled-content>\n                    , and upregulated in\n                    <jats:styled-content style=\"fixed-case\">DMD<\/jats:styled-content>\n                    . It is possible that the absence of dystrophin at\n                    <jats:styled-content style=\"fixed-case\">NMJ<\/jats:styled-content>\n                    s in\n                    <jats:styled-content style=\"fixed-case\">DMD<\/jats:styled-content>\n                    causes neuromuscular transmission defects that aggravate muscle weakness. We studied\n                    <jats:styled-content style=\"fixed-case\">NMJ<\/jats:styled-content>\n                    function in\n                    <jats:italic>mdx<\/jats:italic>\n                    mice (lacking dystrophin) and wild type mice. In addition,\n                    <jats:italic>mdx\/utrn<\/jats:italic>\n                    <jats:sup>+\/\u2212<\/jats:sup>\n                    and\n                    <jats:italic>mdx\/utrn<\/jats:italic>\n                    <jats:sup>\u2212\/\u2212<\/jats:sup>\n                    mice (lacking utrophin) were used to investigate influences of utrophin levels. The three Duchenne mouse models showed muscle weakness when comparatively tested\n                    <jats:italic>in\u00a0vivo<\/jats:italic>\n                    , with\n                    <jats:italic>mdx\/utrn<\/jats:italic>\n                    <jats:sup>\u2212\/\u2212<\/jats:sup>\n                    mice being weakest.\n                    <jats:italic>Ex vivo<\/jats:italic>\n                    muscle contraction and electrophysiological studies showed a reduced safety factor of neuromuscular transmission in all models.\n                    <jats:styled-content style=\"fixed-case\">NMJ<\/jats:styled-content>\n                    s had ~\u00a040% smaller miniature endplate potential amplitudes compared with wild type, indicating postsynaptic sensitivity loss for the neurotransmitter acetylcholine. However, nerve stimulation\u2010evoked endplate potential amplitudes were unchanged. Consequently, quantal content (i.e. the number of acetylcholine quanta released per nerve impulse) was considerably increased. Such a homeostatic compensatory increase in neurotransmitter release is also found at\n                    <jats:styled-content style=\"fixed-case\">NMJ<\/jats:styled-content>\n                    s in myasthenia gravis, where autoantibodies reduce acetylcholine receptors. However, high\u2010rate nerve stimulation induced exaggerated endplate potential rundown. Study of\n                    <jats:styled-content style=\"fixed-case\">NMJ<\/jats:styled-content>\n                    morphology showed that fragmentation of acetylcholine receptor clusters occurred in all models, being most severe in\n                    <jats:italic>mdx\/utrn<\/jats:italic>\n                    <jats:sup>\u2212\/\u2212<\/jats:sup>\n                    mice. Overall, we showed mild \u2018myasthenia\u2010like\u2019 neuromuscular synaptic dysfunction in several Duchenne mouse models, which possibly affects muscle weakness and degeneration.\n                  <\/jats:p>","DOI":"10.1111\/ejn.13249","type":"journal-article","created":{"date-parts":[[2016,4,2]],"date-time":"2016-04-02T16:48:36Z","timestamp":1459615716000},"page":"1623-1635","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":77,"title":["Characterization of neuromuscular synapse function abnormalities in multiple Duchenne muscular dystrophy mouse models"],"prefix":"10.1111","volume":"43","author":[{"given":"Elizabeth M.","family":"van der Pijl","sequence":"first","affiliation":[{"name":"Department of Neurology Leiden University Medical Centre Research Building S5\u2010P P.O. 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