LAUSR

Functions and disease associations Sodium-activated potassium channels (KNa) are opened by high concentrations of Na+ in the cytoplasm. The resulting K+ outflux counteracts the membrane potential change caused by the Na+ influx and regulates neuronal firing patterns. The pores of the KNa channels are either homomers or heteromers, containing four subunits encoded by KCNT1 or KCNT2. The KCNT1 and KCNT2 proteins are ~74% identical, and both proteins consist of six transmembrane domains (S1–S6) and an extended cytosolic region at the C terminus (ISRN Neurosci. 2013, 354262; 2013). The C-terminal region includes two regulators of conductance of K+ (RCK) domains, which serve as Na+ sensors and control the of KNa1.1 channels in the central nervous system remains unclear. Mechanistic understanding of how gain-of-function mutations in KCNT1 cause epilepsy is also lacking.