KCNQ2 and channelopathy: In conclusion, the present results add severe forms of Kv7.2-related epilepsy to the growing list of channelopathies caused by changes in PIP2-dependent regulation1, 59; the recent observation that Kv7 channels are critical determinants of the cortical excitability changes occurring upon dynamic regulation of PIP2 levels31, lends further support to the pathogenetic role of the proposed mechanism in individuals carrying the Kv7.2 R325G variant.