Empson, Ruth M. and Akemann, Walther and Knöpfel, Thomas (2010) The role of the calcium transporter protein plasma membrane calcium ATPase PMCA2 in cerebellar Purkinje neuron function. Functional Neurology; New Trends in Interventional Neurosciences, 25 (3). pp. 153-158. ISSN 1971-3274
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Abstract
Genetic deletion of the plasma membrane calcium ATPase type 2 (PMCA2), a calcium transporter protein, is associated with an overtly ataxic phenotype in mice. PMCA2 is expressed at high levels in cerebellar Purkinje neurons (PNs) where functional integrity is essential for normal cerebellar function. Indeed, loss of PN function accompanies cerebellar ataxia in humans and mouse models. In the ataxic PMCA2 knockout (PMCA2-/-) mouse the ability of the PNs to control their cytosolic calcium levels was severely impaired; basal calcium levels were high and calcium recovery kinetics slow. Whole cell patch clamp recordings from PMCA2-/- PNs revealed that they possessed hyperpolarised membrane potentials, reduced frequency and increased irregularity of spontaneous action potential firing, curtailed complex spikes and sustained calcium-dependent outward K+ currents. We propose that these alterations limit pathological excursions in PN cytosolic calcium as an aid to survival but that they are insufficient to prevent loss of functional cerebellar output
Item Type: | Article |
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Uncontrolled Keywords: | Ataxia, calcium, cerebellum, plasma membrane Ca2+ ATPase |
Subjects: | 600 Tecnologia - Scienze applicate > 610 Medicina e salute (Classificare qui la tecnologia dei servizi medici) > 616 Malattie (classificare qui la Clinica medica, la medicina basata sull'evidenza, la Medicina interna, la Medicina sperimentale) > 616.8 Malattie del sistema nervoso e disturbi mentali (Classificare qui la Neuropsichiatria, la Neurologia) |
Depositing User: | Danilo Dezzi |
Date Deposited: | 09 Jan 2012 17:22 |
Last Modified: | 09 Jan 2012 17:22 |
URI: | http://eprints.bice.rm.cnr.it/id/eprint/3553 |
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