KV7 channelopathies

K V 7 voltage-gated potassium channels, encoded by the KCNQ gene family, have caught increasing interest of the scientific community for their important physiological roles, which are emphasized by the fact that four of the five so far identified members are related to different hereditary diseases. Furthermore, these channels prove to be attractive pharmacological targets for treating diseases characterized by membrane hyperexcitability. K V 7 channels are expressed in brain, heart, thyroid gland, pancreas, inner ear, muscle, stomach, and intestines. They give rise to functionally important potassium currents, reduction of which results in pathologies such as long QT syndrome, diabetes, neonatal epilepsy, neuromyotonia, or progressive deafness. Here, we summarize some key traits of K V 7 channels and review how their molecular deficiencies could explain diverse disease phenotypes. We also assess the therapeutic potential of K V 7 channels; in particular, how the activation of K V 7 channels by the compounds retigabine and R-L3 may be useful for treatment of epilepsy or cardiac arrhythmia.