Although the origin of many SCAs is still uncertain, there is an interesting polyglutamine repeat-encoding gene, Ronin (herein referring to Thap11), located in the genomic region on chromosome 16q22.1 (chr16: 66,095,251 and 74,034,030; hg38) that has been mapped to SCA type 4 (SCA4) disease (Flanigan et al., 1996; Hellenbroich et al., 2003, 2006). Here, THAP11 is linked to spinocerebellar ataxia type 4.