While KCNA1 mutations associated with EA1 are spanned along the whole length of Kv1.1 [6], KCNA1 mutations associated with epilepsy preferentially lie in specific domains of the Kv1.1 protein, such as the S1/S2 helices and the pore domain, from the end of the S4–S5 intracellular linker to the beginning of the C-terminal domain [5,8,37]. Here, KCNA1 is linked to episodic ataxia type 1.