As shown from studies using the kcna1 knock-out mouse and the Kv1.1V408A/+ mouse model of EA1, Kv1.1 channels, which are expressed in myelinated axons at juxtaparanodal regions and at branch points, shunt aberrant depolarization and allow proper neuromuscular transmission [39,40]. The gene discussed is KCNA1; the disease is episodic ataxia type 1.