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Mice deficient for the transcription factor Ebf2 lose a subset of Purkinje cells during development and have a hypotrophic cerebellar cortex. Related motor symptoms and the function of Purkinje cells surviving in the adult have not been studied so far. Ebf2 null mice presented locomotor impairment and a deficiency of motor coordination and motor learning. Ebf2 null Purkinje cells of the anterior lobe, relative to wild-type controls, were patch-clamp recorded in acutely prepared slices. While immature Purkinje cells (10-20 postnatal days) of Ebf2 null mice showed no significant difference relative to wild-types, in the adult they featured a higher input resistance, increased anomalous rectification, decreased first spike latency, higher initial firing frequency, lower voltage threshold and reduced afterhyperpolarizations and post-burst hyperpolarizations. These parameters indicate a difference in the response to both hyperpolarizing and depolarizing stimuli, corresponding to an altered cerebellar cortical output signaling. In contrast, adult climbing fibers attained a normal monoinnervation pattern and the parallel fiber-Purkinje cell synapse showed evoked postsynaptic currents and paired-pulse facilitation functionally indistinguishable from wild-type PCs. These results suggest that the motor deficits exhibited by Ebf2 null mice could be due, at least in part, to an impairment of the firing properties of surviving Purkinje cells. These findings indicate that Ebf2 is important for the development and maintenance of normal Purkinje cell discharge properties. Copyright © 2012 Elsevier Inc. All rights reserved.

Citation

Eriola Hoxha, Raffaella Tonini, Francesca Montarolo, Laura Croci, Gian Giacomo Consalez, Filippo Tempia. Motor dysfunction and cerebellar Purkinje cell firing impairment in Ebf2 null mice. Molecular and cellular neurosciences. 2013 Jan;52:51-61

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PMID: 23000673

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