ATXN3 and Huntington disease: Our recent publication also supports this idea because the presence of mHTT or mutant ataxin-3 in the transcription-linked DNA repair complex dramatically impairs its DNA repair activities, resulting in DNA damage/strand break accumulation and chronic activation of the DDR pathway in HD (Gao et al., 2019) as well as in SCA3 (Gao et al., 2015) respectively.