| Summary: | The Moonwalker (<em>Mwk</em>) mouse is a dominant ataxic mouse model of inherited cerebellar ataxia caused by a gain-of-function mutation in the gene encoding the TRPC3 cation channel. <em>Mwk</em> mice display overt ataxia, impaired Purkinje cell development, altered Purkinje cell excitability and loss of TRPC3-expressing neurons in the cerebellum. Recently, dysregulated mGluR1-TRPC3 signalling has been implicated in multiple human spinocerebellar ataxias. Here, we discuss the behavioural, morphological, and functional changes in <em>Mwk</em> mice with an emphasis on their relevance to the human spinocerebellar ataxias.
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