On the one hand, preventing processing through mutation of polyQ-expanded ataxin-7 mitigated cell death, aggregate formation, and transcriptional interference that is observed in SCA7; prevention of cleavage at D266 also reduced neurodegeneration, extended lifespan, and improved motor performance in SCA7 model mice (Young et al., 2007; Guyenet et al., 2015). Here, ATXN7 is linked to spinocerebellar ataxia 7.