QKI which belongs to the STAR family of the KH domain containing RNA-binding proteins, has been found to affect pre-mRNA splicing, mRNA turnover, mRNA translation, and circular RNA formation; QKI has been implicated in different diseases including ataxia, schizophrenia, and tumorigenesis [3, 16, 41, 42]. Here, QKI is linked to cerebellar ataxia.