In this context, we found that missense mutations in N4-2 that were identified in patients with photosensitive epilepsy (S233L, E271A, and H515P) cause increased intrinsic E3 activity of N4-2 (Fig. 2) and/or N4-2-dependent ubiquitination of Kir4.1 and Cx43 (Fig. 4 C). Here, KCNJ10 is linked to photosensitive epilepsy.