Target intelligence / Profile preview

Potassium voltage-gated channel subfamily E regulatory subunit 2 (KCNE2)

Target
KCNE2
Molecular classification
Ion channel (regulatory/accessory subunit), Voltage-gated potassium channel (beta/ancillary subunit)
01

Overview

KCNE2 (Potassium voltage-gated channel subfamily E regulatory subunit 2) is a small, single-pass transmembrane protein that acts as a beta/ancillary subunit for voltage-gated potassium channel complexes, especially those containing the KCNH2 (hERG) alpha subunit in the heart. It modulates channel gating kinetics and stability, influencing the cardiac repolarization phase and overall electrical activity in multiple tissues, including heart, stomach, thyroid, and brain. KCNE2 dysfunction—via inherited mutations or drug interaction—can precipitate long QT syndrome, familial atrial fibrillation, hypothyroidism, gastric pathology, and various metabolic and neurological disorders. KCNE2's clinical relevance stems from its role in arrhythmia susceptibility and its association with genetic and pharmacological risk factors for cardiovascular events

Other names
MinK-related peptide 1MiRP1LQT6LQT5ATFB4MIRP1Potassium channel subunit beta MiRP1Potassium voltage-gated channel subfamily E member 2Cardiac voltage-gated potassium channel accessory subunit 2Voltage-gated K+ channel subunit MIRP1Potassium channel, voltage gated subfamily E regulatory beta subunit 2Potassium voltage-gated channel, Isk-related family, member 2Minimum potassium ion channel-related peptide 1
02

Mechanism of action

Blockade of hERG (KCNH2)/KCNE2 channel complex by drugs prolongs the cardiac action potential (QT interval), increasing risk for arrhythmia/torsades de pointes Modulation of cardiac repolarization by altering delayed rectifier potassium current (IKr)

03

Biological functions

Regulation of cardiac action potential (repolarization)Control of neurotransmitter releaseEpithelial electrolyte transportSmooth muscle contractionModulation of neuronal excitabilityInsulin secretionRegulation of cell volume
04

Disease associations

Cardiovascular disease (Long QT syndrome, Atrial fibrillation)Cancer (gastric neoplasia, adenocarcinoma)Endocrine disorders (hypothyroidism)Neurological disorders (seizure susceptibility)Metabolic disorders (glucose intolerance, dyslipidemia)
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Safety considerations

Drug-induced QT prolongation and torsades de pointesSudden cardiac death risk in subjects with KCNE2 mutations or variantsIncreased susceptibility to adverse drug effects for drugs that block potassium channels
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Interacting drugs

Dofetilide

5 more in the full profile.

07

Biomarkers

KCNE2 gene mutations for Long QT syndrome riskSNPs in KCNE2 for coronary artery disease risk and statin clinical benefit

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