Cerebellar Ataxia by Enhanced CaV2.1 Currents Is Alleviated by Ca -Dependent K -Channel Activators in Cacna1a Mutant Mice
نویسندگان
چکیده
Zhenyu Gao,1 Boyan Todorov,2 Curtis F. Barrett,2,3 Stijn van Dorp,4 Michel D. Ferrari,3 Arn M.J.M. van den Maagdenberg,2,3 Chris I. De Zeeuw,1,4 and Freek E. Hoebeek1 1Department of Neuroscience, Erasmus Medical Centre, 3000 CA Rotterdam, Netherlands; 2Department of Human Genetics, and 3Department of Neurology, Leiden University Medical Centre, 2300 RC Leiden, Netherlands; 4Netherlands Institute for Neuroscience, Royal Academy of Arts & Sciences (KNAW), 1105 BA Amsterdam, Netherlands
منابع مشابه
Spinocerebellar ataxia type 6 knockin mice develop a progressive neuronal dysfunction with age-dependent accumulation of mutant CaV2.1 channels.
Spinocerebellar ataxia type 6 (SCA6) is a neurodegenerative disorder caused by CAG repeat expansions within the voltage-gated calcium (Ca(V)) 2.1 channel gene. It remains controversial whether the mutation exerts neurotoxicity by changing the function of Ca(V)2.1 channel or through a gain-of-function mechanism associated with accumulation of the expanded polyglutamine protein. We generated thre...
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