ATXN7 and spinocerebellar ataxia 7: In addition, SCA7-266Q mice exhibit a rapid onset of disease consistent with early-onset SCA7: although largely asymptomatic at 4 weeks, they show severe symptoms by 8 weeks, as previous studies of this mouse model have also reported (Niu et al., 2018; Noma et al., 2012; Stoyas et al., 2020; Yoo et al., 2003).