A molecular switch in RCK2 triggers sodium-dependent activation of K1.1 (KCNT1) potassium channels.

The Na-activated K channel K1.1, encoded by the KCNT1 gene, is an important regulator of neuronal excitability. How intracellular Na ions bind and increase channel activity is not well understood.

Modulation and Regulation of Canonical Transient Receptor Potential 3 (TRPC3) Channels.

Canonical transient receptor potential 3 (TRPC3) channel is a non-selective cation permeable channel that plays an essential role in calcium signalling. TRPC3 is highly expressed in the brain and also found in endocrine tissues and smooth muscle cells. The channel is activated directly by binding of diacylglycerol downstream of G-protein coupled receptor activation.

Targeting K1.1 channels in KCNT1-associated epilepsy.

Gain-of-function (GOF) pathogenic variants of KCNT1, the gene encoding the largest known potassium channel subunit, K1.1, are associated with developmental and epileptic encephalopathies accompanied by severe psychomotor and intellectual disabilities. Blocking hyperexcitable K1.

Structure-Based Identification and Characterization of Inhibitors of the Epilepsy-Associated K1.1 (KCNT1) Potassium Channel.

Drug-resistant epileptic encephalopathies of infancy have been associated with KCNT1 gain-of-function mutations, which increase the activity of K1.1 sodium-activated potassium channels. Pharmacological inhibition of hyperactive K1.