In conclusion, functional alteration of mGluR1 activity has been documented in different SCA mouse model, while, in human patients, several forms of cerebellar ataxia result from mutations in genes in this pathway, which suggests that disruption of mGluR1 signaling and downstream Ca2+ homeostasis form a common pathological mechanism underlying SCAs [21]. This evidence concerns the gene GRM1 and autosomal dominant cerebellar ataxia.