Among dominantly inherited CAs, CAG repeat expansions were firstly described in SCA1 in 1993, and subsequently in dentatorubral-pallidoluysian atrophy (DRPLA), SCA3, SCA2, SCA6, SCA7, SCA12, and SCA17 [5]. Here, ATXN3 is linked to dentatorubral-pallidoluysian atrophy.