Missense mutations of Itpr1 have been found in patients suffering from spinocerebellar ataxia (Barresi et al., 2017; Hara et al., 2008), and cerebellum-specific deletion of Itpr1 in mice induces severe ataxia and synaptic loss (Egorova et al., 2016; Kasumu et al., 2012), indicating the importance of ER calcium release for neuronal health. This evidence concerns the gene ITPR1 and cerebellar ataxia.