To assess the functional consequences of the amino acid variant within parkin on the molecular pathogenesis of MJD, that may explain the observed decrease in the AAO of patients, we generated N-terminally 6xMyc-tagged parkin constructs with and without the respectively encoded V380L variant (for a schematic representation, see Supplementary Fig. S5a). Here, PRKN is linked to Spinocerebellar ataxia type 3.