Given that the deubiquitinase ATXN3 is present in the TCR complex, interacts with mHTT, and is sequestered in polyQ aggregates in HD brain, we postulated that compromised ATXN3 activity might increase ubiquitination and decrease levels of TCR components, adversely impacting complex functionality and transcription. The gene discussed is ATXN3; the disease is Huntington disease.