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The Journal of Neuroscience, June 15, 1998, 18(12):4482-4489
Altered Calcium Channel Currents in Purkinje Cells of the Neurological Mutant Mouse leaner
Nancy M. Lorenzon1, Cathleen M. Lutz2, Wayne N. Frankel2, and Kurt G. Beam1 1 Department of Anatomy and Neurobiology, Colorado State University, Fort Collins, Colorado 80523, and 2 The Jackson Laboratory, Bar Harbor, Maine 04609
Mutations of the
1A calcium channel subunit have been shown to cause such human neurological diseases as familial hemiplegic migraine, episodic ataxia-2, and spinocerebellar ataxia 6 and also to cause the murine neurological phenotypes of tottering and leaner. The leaner phenotype is recessive and characterized by ataxia with cortical spike and wave discharges (similar to absence epilepsy in humans) and a gradual degeneration of cerebellar Purkinje and granule cells. The mutation responsible is a single-base substitution that produces truncation of the normal open reading frame beyond repeat IV and expression of a novel C-terminal sequence. Here, we have used whole-cell recordings to determine whether the leaner mutation alters calcium channel currents in cerebellar Purkinje cells, both because these cells are profoundly affected in leaner mice and because they normally express high levels of
-agatoxin-IVA. In Purkinje cells from homozygous leaner mice, this
Key words: calcium channel; cerebellum; Purkinje cell; neurological disorders; mutant mice;
Copyright © 1998 Society for Neuroscience 0270-6474/98/18124482-08$05.00/0
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