<b>Abbreviations:</b> MJDM: achado-Joseph disease; SCA3: spinocerebellar ataxia type 3; PolyQ: polyglutamine; CNS: central nervous system; CFTR: cystic fibrosis transmembrane conductance regulator; CF: cystic fibrosis; UIMs: ubiquitin-interacting motifs; MEM: Minimum Essential Medium; FBS: fetal bovine serum; P/S: penicillin/streptomycin; siRNA: small interfering RNA; BSA: bovine serum albumin; Co-IP: Co-immunoprecipitation; LPS: lipopolysaccharide; WT-CFTR: wild-type CFTR; CHX: Cycloheximide; 3-MA: 3-Methyladenine; IF: Immunofluorescence; IB: immunoblot; Ub: ubiquitin. Here, ATXN3 is linked to cystic fibrosis.