The previously observed CCCP-induced lowering of MFN2 and VDAC1 levels for parkin V380L were recapitulated upon ataxin-3 148Q but not ataxin-3 15Q co-expression, confirming the relevance of the parkin variant for MJD (Fig. 4a, b). Here, ATXN3 is linked to Spinocerebellar ataxia type 3.