Potassium channelopathies associated with epilepsy-related syndromes and directions for therapeutic intervention

VK Gribkoff, RJ Winquist - Biochemical Pharmacology, 2023 - Elsevier
A number of mutations to members of several CNS potassium (K) channel families have
been identified which result in rare forms of neonatal onset epilepsy, or syndromes of which …

Structures illuminate cardiac ion channel functions in health and in long QT syndrome

KR Brewer, G Kuenze, CG Vanoye… - Frontiers in …, 2020 - frontiersin.org
The cardiac action potential is critical to the production of a synchronized heartbeat. This
electrical impulse is governed by the intricate activity of cardiac ion channels, among them …

[HTML][HTML] A calmodulin C-lobe Ca2+-dependent switch governs Kv7 channel function

A Chang, F Abderemane-Ali, GL Hura, ND Rossen… - Neuron, 2018 - cell.com
Summary Kv7 (KCNQ) voltage-gated potassium channels control excitability in the brain,
heart, and ear. Calmodulin (CaM) is crucial for Kv7 function, but how this calcium sensor …

Structure and physiological function of the human KCNQ1 channel voltage sensor intermediate state

KC Taylor, PW Kang, P Hou, ND Yang, G Kuenze… - Elife, 2020 - elifesciences.org
Voltage-gated ion channels feature voltage sensor domains (VSDs) that exist in three
distinct conformations during activation: resting, intermediate, and activated. Experimental …

A PIP2 substitute mediates voltage sensor-pore coupling in KCNQ activation

Y Liu, X Xu, J Gao, MM Naffaa, H Liang, J Shi… - Communications …, 2020 - nature.com
Abstract KCNQ family K+ channels (KCNQ1-5) in the heart, nerve, epithelium and ear
require phosphatidylinositol 4, 5-bisphosphate (PIP2) for voltage dependent activation …

Phosphatidylinositol 4, 5-bisphosphate (PIP2) regulates KCNQ3 K+ channels by interacting with four cytoplasmic channel domains

FS Choveau, V De la Rosa, SM Bierbower… - Journal of Biological …, 2018 - ASBMB
Phosphatidylinositol 4, 5-bisphosphate (PIP 2) in the plasma membrane regulates the
function of many ion channels, including M-type (potassium voltage-gated channel …

[HTML][HTML] Reduced axonal surface expression and phosphoinositide sensitivity in Kv7 channels disrupts their function to inhibit neuronal excitability in Kcnq2 epileptic …

EC Kim, J Zhang, W Pang, S Wang, KY Lee… - Neurobiology of …, 2018 - Elsevier
Neuronal K v 7/KCNQ channels are voltage-gated potassium channels composed of K v
7.2/KCNQ2 and K v 7.3/KCNQ3 subunits. Enriched at the axonal membrane, they potently …

Ion current profiles in canine ventricular myocytes obtained by the “onion peeling” technique

B Horváth, D Kiss, C Dienes, T Hézső, Z Kovács… - Journal of molecular and …, 2021 - Elsevier
The profiles of ion currents during the cardiac action potential can be visualized by the
action potential voltage clamp technique. To obtain multiple ion current data from the same …

The crossroad of ion channels and calmodulin in disease

J Urrutia, A Aguado, A Muguruza-Montero… - International journal of …, 2019 - mdpi.com
Calmodulin (CaM) is the principal Ca2+ sensor in eukaryotic cells, orchestrating the activity
of hundreds of proteins. Disease causing mutations at any of the three genes that encode …

Voltage-gated potassium channels KCNQs: structures, mechanisms, and modulations

Y Huang, D Ma, Z Yang, Y Zhao, J Guo - Biochemical and Biophysical …, 2023 - Elsevier
KCNQ (Kv7) channels are voltage-gated, phosphatidylinositol 4, 5-bisphosphate-(PIP 2-)
modulated potassium channels that play essential roles in regulating the activity of neurons …