{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T19:06:50Z","timestamp":1790276810133,"version":"4.1.0"},"reference-count":49,"publisher":"Society for Neuroscience","issue":"48","license":[{"start":{"date-parts":[[2007,5,29]],"date-time":"2007-05-29T00:00:00Z","timestamp":1180396800000},"content-version":"vor","delay-in-days":181,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-sa\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. Neurosci."],"published-print":{"date-parts":[[2006,11,29]]},"abstract":"<jats:p>The mouse mutant ducky and its allele ducky<jats:italic><jats:sup>2J<\/jats:sup><\/jats:italic>represent a model for absence epilepsy characterized by spike-wave seizures and cerebellar ataxia. These mice have mutations in<jats:italic>Cacna2d2<\/jats:italic>, which encodes the \u03b1<jats:sub>2<\/jats:sub>\u03b4-2 calcium channel subunit. Of relevance to the ataxic phenotype, \u03b1<jats:sub>2<\/jats:sub>\u03b4-2 mRNA is strongly expressed in cerebellar Purkinje cells (PCs). The<jats:italic>Cacna2d2<jats:sup>du2J<\/jats:sup><\/jats:italic>mutation results in a 2 bp deletion in the coding region and a complete loss of \u03b1<jats:sub>2<\/jats:sub>\u03b4-2 protein. Here we show that<jats:italic>du<jats:sup>2J<\/jats:sup>\/du<jats:sup>2J<\/jats:sup><\/jats:italic>mice have a 30% reduction in somatic calcium current and a marked fall in the spontaneous PC firing rate at 22\u00b0C, accompanied by a decrease in firing regularity, which is not affected by blocking synaptic input to PCs. At 34\u00b0C,<jats:italic>du<jats:sup>2J<\/jats:sup>\/du<jats:sup>2J<\/jats:sup><\/jats:italic>PCs show no spontaneous intrinsic activity.<jats:italic>Du<jats:sup>2J<\/jats:sup>\/du<jats:sup>2J<\/jats:sup><\/jats:italic>mice also have alterations in the cerebellar expression of several genes related to PC function. At postnatal day 21, there is an elevation of tyrosine hydroxylase mRNA and a reduction in tenascin-C gene expression. Although<jats:italic>du<jats:sup>2J<\/jats:sup><\/jats:italic>\/+ mice have a marked reduction in \u03b1<jats:sub>2<\/jats:sub>\u03b4-2 protein, they show no fall in PC somatic calcium currents or increase in cerebellar tryrosine hydroxylase gene expression. However,<jats:italic>du<jats:sup>2J<\/jats:sup><\/jats:italic>\/+ PCs do exhibit a significant reduction in firing rate, correlating with the reduction in \u03b1<jats:sub>2<\/jats:sub>\u03b4-2. A hypothesis for future study is that effects on gene expression occur as a result of a reduction in somatic calcium currents, whereas effects on PC firing occur as a long-term result of loss of \u03b1<jats:sub>2<\/jats:sub>\u03b4-2 and\/or a reduction in calcium currents and calcium-dependent processes in regions other than the soma.<\/jats:p>","DOI":"10.1523\/jneurosci.3080-06.2006","type":"journal-article","created":{"date-parts":[[2006,11,29]],"date-time":"2006-11-29T22:55:24Z","timestamp":1164840924000},"page":"12576-12586","source":"Crossref","is-referenced-by-count":64,"title":["The ducky<i><sup>2J<\/sup><\/i>Mutation in<i>Cacna2d2<\/i>Results in Reduced Spontaneous Purkinje Cell Activity and Altered Gene Expression"],"prefix":"10.1523","volume":"26","author":[{"given":"Roberta","family":"Donato","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karen 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