ATXN3 and Spinocerebellar ataxia type 3: In 304Q knock-in spinocerebellar ataxia type 3 (SCA3) mouse model, serum NfL and pNfH are elevated at the pre-symptomatic stage of 6 months of age and correlate with ataxin 3 aggregation and Purkinje cell loss in the brain (Wilke et al., 2020).