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Notably, partially blocking sodium pumps in the cerebellum was necessary and sufficient for induction of dystonia. Thus, a key question that remains is how partially blocking sodium pumps in the cerebellum induces dystonia.<jats:italic>In vivo<\/jats:italic>recordings from dystonic mice revealed abnormal high-frequency bursting activity in neurons of the deep cerebellar nuclei (DCN), which comprise the bulk of cerebellar output. In the same mice, Purkinje cells, which provide strong inhibitory drive to DCN cells, also fired in a similarly erratic manner.<jats:italic>In vitro<\/jats:italic>studies demonstrated that Purkinje cells are highly sensitive to sodium pump dysfunction that alters the intrinsic pacemaking of these neurons, resulting in erratic burst firing similar to that identified<jats:italic>in vivo<\/jats:italic>. This abnormal firing abates when sodium pump function is restored and dystonia caused by partial block of sodium pumps can be similarly alleviated. These findings suggest that persistent high-frequency burst firing of cerebellar neurons caused by sodium pump dysfunction underlies dystonia in this model of DYT12.<\/jats:p>","DOI":"10.1523\/jneurosci.1409-14.2014","type":"journal-article","created":{"date-parts":[[2014,9,8]],"date-time":"2014-09-08T22:26:44Z","timestamp":1410215204000},"page":"11723-11732","source":"Crossref","is-referenced-by-count":121,"title":["Abnormal High-Frequency Burst Firing of Cerebellar Neurons in Rapid-Onset Dystonia-Parkinsonism"],"prefix":"10.1523","volume":"34","author":[{"given":"Rachel","family":"Fremont","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D. 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