This mild accumulation is compared to neurons in a mouse model of Alzheimer’s, JNPL3(P301L), which expresses a mutated form of Tau (Lewis et al., 2000) and is therefore positive for accumulation of phosphorylated Tau (Figure 4—figure supplement 2B). Thus, dysfunction of PCs after loss of Ttbk2, accompanied by Tau accumulation outside of the cerebellum, recapitulates currently described SCA11 phenotypes. This evidence concerns the gene TTBK2 and spinocerebellar ataxia type 11.