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It is associated with mutations in the<jats:italic>CLN3<\/jats:italic>gene, causing loss of function of CLN3 protein and degeneration of cerebellar and retinal neurons. It has been proposed that changes in granule cell AMPA-type glutamate receptors (AMPARs) contribute to the cerebellar dysfunction. In this study, we compared AMPAR properties and synaptic transmission in cerebellar granule cells from wild-type and<jats:italic>Cln3<\/jats:italic>knock-out mice. In<jats:italic>Cln3<\/jats:italic><jats:sup>\u0394<jats:italic>ex1\u20136<\/jats:italic><\/jats:sup>cells, the amplitude of AMPA-evoked whole-cell currents was unchanged. Similarly, we found no change in the amplitude, kinetics, or rectification of synaptic currents evoked by individual quanta, or in their underlying single-channel conductance. We found no change in cerebellar expression of GluA2 or GluA4 protein. By contrast, we observed a reduced number of quantal events following mossy-fiber stimulation in Sr<jats:sup>2+<\/jats:sup>, altered short-term plasticity in conditions of reduced extracellular Ca<jats:sup>2+<\/jats:sup>, and reduced mossy fiber vesicle number. Thus, while our results suggest early presynaptic changes in the<jats:italic>Cln3<\/jats:italic><jats:sup>\u0394<\/jats:sup><jats:italic><jats:sup>ex1\u20136<\/jats:sup><\/jats:italic>mouse model of juvenile Batten disease, they reveal no evidence for altered postsynaptic AMPARs.<\/jats:p>","DOI":"10.1523\/eneuro.0387-17.2018","type":"journal-article","created":{"date-parts":[[2018,4,20]],"date-time":"2018-04-20T15:50:12Z","timestamp":1524239412000},"page":"ENEURO.0387-17.2018","source":"Crossref","is-referenced-by-count":7,"title":["Altered Cerebellar Short-Term Plasticity but No Change in Postsynaptic AMPA-Type Glutamate Receptors in a Mouse Model of Juvenile Batten Disease"],"prefix":"10.1523","volume":"5","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4109-7956","authenticated-orcid":false,"given":"Dorota","family":"Studniarczyk","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth L.","family":"Needham","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hannah M.","family":"Mitchison","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9918-0376","authenticated-orcid":false,"given":"Mark","family":"Farrant","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0831-8326","authenticated-orcid":false,"given":"Stuart G.","family":"Cull-Candy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"393","published-online":{"date-parts":[[2018,4,20]]},"reference":[{"key":"2023041302124095000_5.2.ENEURO.0387-17.2018.1","doi-asserted-by":"publisher","DOI":"10.1113\/jphysiol.1996.sp021578"},{"key":"2023041302124095000_5.2.ENEURO.0387-17.2018.2","doi-asserted-by":"publisher","DOI":"10.1002\/brb3.401"},{"key":"2023041302124095000_5.2.ENEURO.0387-17.2018.3","doi-asserted-by":"publisher","DOI":"10.1007\/BF00607283"},{"key":"2023041302124095000_5.2.ENEURO.0387-17.2018.4","doi-asserted-by":"publisher","DOI":"10.18637\/jss.v067.i01"},{"key":"2023041302124095000_5.2.ENEURO.0387-17.2018.5","doi-asserted-by":"publisher","DOI":"10.1016\/j.tins.2010.03.003"},{"key":"2023041302124095000_5.2.ENEURO.0387-17.2018.6","doi-asserted-by":"publisher","DOI":"10.1016\/0896-6273(95)90049-7"},{"key":"2023041302124095000_5.2.ENEURO.0387-17.2018.7","doi-asserted-by":"publisher","DOI":"10.1016\/S1096-7192(02)00201-9"},{"key":"2023041302124095000_5.2.ENEURO.0387-17.2018.8","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2016.12.022"},{"key":"2023041302124095000_5.2.ENEURO.0387-17.2018.9","doi-asserted-by":"publisher","DOI":"10.1016\/0896-6273(92)90120-3"},{"key":"2023041302124095000_5.2.ENEURO.0387-17.2018.10","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbadis.2015.04.027"},{"key":"2023041302124095000_5.2.ENEURO.0387-17.2018.11","doi-asserted-by":"publisher","DOI":"10.1038\/nn.3974"},{"key":"2023041302124095000_5.2.ENEURO.0387-17.2018.12","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M114.621706"},{"key":"2023041302124095000_5.2.ENEURO.0387-17.2018.13","doi-asserted-by":"publisher","DOI":"10.1152\/jn.90404.2008"},{"key":"2023041302124095000_5.2.ENEURO.0387-17.2018.14","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuropharm.2006.12.008"},{"key":"2023041302124095000_5.2.ENEURO.0387-17.2018.15","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/11.22.2709"},{"key":"2023041302124095000_5.2.ENEURO.0387-17.2018.16","doi-asserted-by":"publisher","DOI":"10.1007\/s11910-013-0366-z"},{"key":"2023041302124095000_5.2.ENEURO.0387-17.2018.17","doi-asserted-by":"publisher","DOI":"10.1113\/jphysiol.1988.sp017117"},{"key":"2023041302124095000_5.2.ENEURO.0387-17.2018.18","doi-asserted-by":"publisher","DOI":"10.1016\/j.conb.2006.05.012"},{"key":"2023041302124095000_5.2.ENEURO.0387-17.2018.19","doi-asserted-by":"crossref","unstructured":"Eccles JC , Ito M , Szentagothai J (1967) The cerebellum as a neuronal machine. 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