Mutations in the KCNMA1 gene that encode the (α) subunit of BK channels are associated with a wide range of diseases, including epilepsy, dyskinesis, autism, multiple congenital abnormalities, developmental delay, intellectual disability, axial hypotonia, ataxia, cerebral and cerebellar atrophy, bone thickening, and tortuosity of arteries (Wang et al., 2009; Yang et al., 2010; Bailey et al., 2019; Liang et al., 2019; Cui, 2021; Miller et al., 2021). The gene discussed is KCNMA1; the disease is Ataxia.