Ibrahim MF., Becker EBE.
The Moonwalker (Mwk) 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. Mwk 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 Mwk mice with an emphasis on their relevance to the human spinocerebellar ataxias.