Cardiomyocytes are endowed with a complex repertoire of ion channels, responsible for the
generation of action potentials (APs), travelling waves of electrical excitation, propagating …

Cardiac action potentials are characterized by an initial depolarization followed by a
prolonged depolarization, or plateau phase, before a return to the resting potential. In these …

Mutations that induce loss of function (LOF) or dysfunction of the human KCNQ1 channel are
responsible for susceptibility to a life-threatening heart rhythm disorder, the congenital long …

Voltage-gated potassium 7.1 (Kv7. 1) channel and KCNE1 protein coassembly forms the
slow potassium current IKS that repolarizes the cardiac action potential. The physiological …

Long QT syndrome type 1 (LQT1) is a subtype of a congenital cardiac syndrome caused by
mutation in the KCNQ1 gene, which encodes the α-subunit of the slow component of …

Background: The Kv7 (KCNQ) subfamily of voltage-gated potassium channels consists of 5
members (Kv7. 1-5) each showing characteristic tissue distribution and physiological roles …

Ubiquitylation controls protein function and degradation. Therefore, ubiquitin ligases need to
be tightly controlled. We discovered an evolutionarily conserved allosteric restraint …

Background Ion channels play crucial roles in cellular biology, physiology, and
communication including sensory perception. Voltage-gated potassium (Kv) channels …

In the heart, co-assembly of Kv7. 1 with KCNE1 produces the slow IKS potassium current,
which repolarizes the cardiac action potential and mutations in human Kv7. 1 and KCNE1 …

Genetic testing for inherited arrhythmias and discriminating pathogenic or benign variants
from variants of unknown significance (VUS) is essential for gene-based medicine. KCNQ1 …