KCNQ potassium channels: physiology, pathophysiology, and pharmacology

J Robbins - Pharmacology & therapeutics, 2001 - Elsevier
KCNQ genes encode a growing family of six transmembrane domains, single pore-loop, K+
channel α-subunits that have a wide range of physiological correlates. KCNQ1 (KvLTQ1) is …

Neuronal KCNQ potassium channels: physislogy and role in disease

TJ Jentsch - Nature Reviews Neuroscience, 2000 - nature.com
Humans have over 70 potassium channel genes, but only some of these have been linked
to disease. In this respect, the KCNQ family of potassium channels is exceptional: mutations …

A constitutively open potassium channel formed by KCNQ1 and KCNE3

BÈC Schroeder, S Waldegger, S Fehr, M Bleich… - Nature, 2000 - nature.com
Mutations in all four known KCNQ potassium channel α-subunit genes lead to human
diseases,,,,,. KCNQ1 (KvLQT1) interacts with the β-subunit KCNE1 (IsK, minK) to form the …

Differential expression of kcnq2 splice variants: implications to m current function during neuronal development

JS Smith, CA Iannotti, P Dargis… - Journal of …, 2001 - Soc Neuroscience
The KCNQ family of K+ channels has been implicated in several cardiac and neurological
disease pathologies. KCNQ2 (Q2) is a brain-derived gene, which in association with KCNQ3 …

[HTML][HTML] KCNQ4, a novel potassium channel expressed in sensory outer hair cells, is mutated in dominant deafness

C Kubisch, BC Schroeder, T Friedrich, B Lütjohann… - Cell, 1999 - cell.com
Potassium channels regulate electrical signaling and the ionic composition of biological
fluids. Mutations in the three known genes of the KCNQ branch of the K+ channel gene …

KCNQ4, a K+ channel mutated in a form of dominant deafness, is expressed in the inner ear and the central auditory pathway

T Kharkovets, JP Hardelin… - Proceedings of the …, 2000 - National Acad Sciences
Mutations in the potassium channel gene KCNQ4 underlie DFNA2, an autosomal dominant
form of progressive hearing loss in humans. In the mouse cochlea, the transcript has been …

Functional expression of two KvLQT1-related potassium channels responsible for an inherited idiopathic epilepsy

WP Yang, PC Levesque, WA Little, ML Conder… - Journal of Biological …, 1998 - ASBMB
Benign familial neonatal convulsions (BFNC), a class of idiopathic generalized epilepsy, is
an autosomal dominantly inherited disorder of newborns. BFNC has been linked to …

A carboxy‐terminal domain determines the subunit specificity of KCNQ K+ channel assembly

M Schwake, TJ Jentsch, T Friedrich - EMBO reports, 2003 - embopress.org
Mutations in KCNQ K+ channel genes underlie several human pathologies. KCNQ α‐
subunits form either homotetramers or hetero‐oligomers with a restricted subset of other …

Pharmacological activation of normal and arrhythmia-associated mutant KCNQ1 potassium channels

G Seebohm, M Pusch, J Chen… - Circulation research, 2003 - Am Heart Assoc
KCNQ1 α-subunits coassemble with KCNE1 β-subunits to form channels that conduct the
slow delayed rectifier K+ current (I Ks) important for repolarization of the cardiac action …

Molecular cloning and functional expression of KCNQ5, a potassium channel subunit that may contribute to neuronal M-current diversity

C Lerche, CR Scherer, G Seebohm, C Derst… - Journal of Biological …, 2000 - ASBMB
We have isolated KCNQ5, a novel human member of the KCNQ potassium channel gene
family that is differentially expressed in subregions of the brain and in skeletal muscle. When …