BK channels are activated by the P/Q-type calcium channels (Walter et al., 2006), which are mutated in episodic ataxia 2 (EA2) (Guida et al., 2001; Mantuano et al., 2004; Tonelli et al., 2006; Walter et al., 2006) and spinocerebellar ataxia 6 (SCA6) (Ishikawa et al., 1997; Bürk et al., 2014). Here, KCNMA1 is linked to spinocerebellar ataxia type 6.