TBP is an important general transcription factor that also plays a role in the mechanism of other polyQ disorders (SCA1, SCA2, SCA3, Huntington’s disease, and dentatorubral-pallidoluysian atrophy) by sequestering in the polyQ-pathological aggregates of those diseases [15,16]. Here, ATXN3 is linked to dentatorubral-pallidoluysian atrophy.