A deubiquitinating enzyme of the ATAXINS family. It functions in protein homeostasis, GENETIC TRANSCRIPTION; CYTOSKELETON regulation, and MYOGENESIS. CAG TRINUCLEOTIDE REPEAT EXPANSION in the Ataxin-3 gene coding region is associated with spinocerebellar ataxia-3 (MACHADO-JOSEPH DISEASE).

Synonyms: Ataxin-3 Protein, EC 3.4.19.12, Ataxin 3, Ataxin-3, Machado Joseph Disease Protein, SCA3 Protein, Ataxin 3 Protein, ATXN3 Protein, Spinocerebellar Ataxia 3 Protein, Machado-Joseph Disease Protein