Dina Simkin, Kelly A Marshall ... Evangelos Kiskinis
An inducedpluripotent stem cell (iPSC)-based model of KCNQ2-associated developmental epileptic encephalopathy suggests that disease is driven by dyshomeostaic neuronal mechanisms that are downstream of loss of M-current.
Among three new models for KCNMA1 channelopathy, the most severe gain-of-function variant (Kcnma1N999S/WT) displays a particular type of immobilizing paroxysmal dyskinesia observed in patients, including amphetamine responsiveness.
An integrative structural biology approach provides refined models of the KCNQ1-KCNE1 channel complex, which propose a new mechanism to explain how KCNE1 modulates KCNQ1 channel activation.
Keenan C Taylor, Po Wei Kang ... Charles R Sanders
The intermediate state conformation of the human KCNQ1 potassium channel voltage sensor domain was determined, validated, and shown to be conductive under physiological conditions.
Benjamin M Zemel, Alexander A Nevue ... Henrique von Gersdorff
Molecular and electrophysiological evidence shows that Kv3 subunits contribute critically to ultrashort action potential waveforms and high-frequency firing in large projection neurons in zebra finch motor nuclei controlling song production and somatic movements.
Characterization of an extra-membrane site in IKs formed by KCNQ1 S1, Pore and S6 domains and the near extracellular region of KCNE1, which forms a binding pocket for channel activators.
Bernadette O'Donovan, Adewale Adeluyi ... Pavel I Ortinski
Low effort-based motivation to pursue naturally rewarding stimuli can be increased pharmacologically, by targeting inactivation of a single voltage-gated potassium channel within the mesolimbic dopamine reward system.
Guided by the available cryo-EM structure of the KCNQ1-KCNE3 ion channel complex, the critical role of the S1 segment of the voltage-sensor domain in the gating modulation by KCNE3 is revealed.
ML277 exclusively enhances the AO state voltage-sensing domain (VSD)-pore coupling of KCNQ1 channels, providing an effective tool to investigate the voltge-dependent gating and new strategies for treating long QT syndrome.
