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Spinocerebellar ataxia type 6 (SCA6) is caused by polyglutamine expansion in P/Q-type Ca 2+ channels (Ca v 2.1) and is characterized by predominant degeneration of cerebellar Purkinje cells. To characterize the Ca v 2.1 channel with an SCA6 mutation in cerebellar Purkinje cells, we have generated knock-in mouse models that express human Ca v 2.1 with 28 polyglutamine...
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