{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T16:44:14Z","timestamp":1787503454551,"version":"build-2736575974"},"reference-count":95,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2024,10,16]],"date-time":"2024-10-16T00:00:00Z","timestamp":1729036800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Eur J of Neuroscience"],"published-print":{"date-parts":[[2024,11]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Patients with Duchenne muscular dystrophy (DMD) commonly show specific cognitive deficits in addition to a severe muscle impairment caused by the absence of dystrophin expression in skeletal muscle. These cognitive deficits have been related to the absence of dystrophin in specific regions of the central nervous system, notably cerebellar Purkinje cells (PCs). Dystrophin has recently been involved in GABA\n                    <jats:sub>A<\/jats:sub>\n                    receptors clustering at postsynaptic densities, and its absence, by disrupting this clustering, leads to decreased inhibitory input to PC. We performed an in vivo electrophysiological study of the dystrophin\u2010deficient muscular dystrophy X\u2010linked (\n                    <jats:italic>mdx<\/jats:italic>\n                    ) mouse model of DMD to compare PC firing and local field potential (LFP) in alert\n                    <jats:italic>mdx<\/jats:italic>\n                    and control C57Bl\/10 mice. We found that the absence of dystrophin is associated with altered PC firing and the emergence of fast (~160\u2013200\u2009Hz) LFP oscillations in the cerebellar cortex of alert\n                    <jats:italic>mdx<\/jats:italic>\n                    mice. These abnormalities were not related to the disrupted expression of calcium\u2010binding proteins in cerebellar PC. We also demonstrate that cerebellar long\u2010term depression is altered in alert\n                    <jats:italic>mdx<\/jats:italic>\n                    mice. Finally,\n                    <jats:italic>mdx<\/jats:italic>\n                    mice displayed a force weakness, mild impairment of motor coordination and balance during behavioural tests. These findings demonstrate the existence of cerebellar dysfunction in\n                    <jats:italic>mdx<\/jats:italic>\n                    mice. A similar cerebellar dysfunction may contribute to the cognitive deficits observed in patients with DMD.\n                  <\/jats:p>","DOI":"10.1111\/ejn.16566","type":"journal-article","created":{"date-parts":[[2024,10,17]],"date-time":"2024-10-17T01:11:42Z","timestamp":1729127502000},"page":"6470-6489","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Cerebellar dysfunction in the\n                    <i>mdx<\/i>\n                    mouse model of Duchenne muscular dystrophy: An electrophysiological and behavioural study"],"prefix":"10.1111","volume":"60","author":[{"given":"Cynthia","family":"Prigogine","sequence":"first","affiliation":[{"name":"Laboratory of Neurophysiology and Movement Biomechanics Universit\u00e9 Libre de Bruxelles  Brussels Belgium"},{"name":"Laboratory of Electrophysiology Universit\u00e9 de Mons  Mons Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Javier 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