In vivo pull-down experiments using HEK293 cells showed reduced binding toward the E4B and enhanced binding toward ataxin 3, thus resembling the accumulation of mutant ataxin 3 on p97 in spinocerebellar ataxia type 3 (Fernández-Sáiz and Buchberger, 2010). This evidence concerns the gene ATXN3 and Spinocerebellar ataxia type 3.