Even though HR23B and proteasome subunits are sequestered into intranuclear inclusions of ataxin-3 in SCA3 patients brain tissue [6, 39], ataxin-3 and 20S do not show an aberrant localization in C9FTD patients post-mortem brain tissue. This evidence concerns the gene ATXN3 and Spinocerebellar ataxia type 3.