In the context of polyQ disease pathology, we revealed that nuclear PQBP3/NOL7 deficiency in Purkinje cells of the SCA1 mouse model (Sca1154Q/2Q) in which an expanded repeat of 154 CAGs was targeted into the mouse Ataxin-1 (Atxn1) locus (Watase et al, 2002) is linked to their senescent phenotype. This evidence concerns the gene NOL7 and spinocerebellar ataxia type 1.