KCNE2

Chr 21q22.11

potassium voltage-gated channel subfamily E regulatory subunit 2

Aliases:: MiRP1, LQT6
MANE:: ENST00000290310.4

OMIM: 603796 ↗NCBI: 9992 ↗Ensembl ↗

Annotations refreshed 9 hours ago.

Predicted protein structure

AlphaFold predicted structure

KCNE2 · Q9Y6J6

v62025-08-01

Predicted Aligned Error matrix for KCNE2 — diagonal shows residue index, colour shows pairwise prediction confidence (lower is better) — Predicted Aligned Error

Mean pLDDT

78.3/ 100

Confident

123 residues

Confidence breakdown

Very high(≥ 90)29%
Confident(70–90)37%
Low(50–70)33%
Very low(< 50)1%

Open interactive 3D viewer

AlphaFold (Jumper et al., 2021) · CC BY 4.0

Clinical relevance (Genomics England PanelApp)

Moderate Evidence (Amber)

Long QT syndrome
MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Short QT syndrome
MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted

Disease associations (Open Targets)

Romano-Ward syndrome
0.74
familial atrial fibrillation
0.61
familial long QT syndrome
0.39
coronary artery disorder
0.29
myocardial infarction
0.29
coronary atherosclerosis
0.27
cardiac arrhythmia
0.27
heart disorder
0.25
aneurysm
0.25
Iron deficiency anemia
0.24

Score is the Open Targets composite evidence score (0-1). Higher = stronger gene-disease association.

Protein function (UniProt)

Potassium voltage-gated channel subfamily E member 2

Ancillary protein that functions as a regulatory subunit of the voltage-gated potassium (Kv) channel complex composed of pore-forming and potassium-conducting alpha subunits and of regulatory beta subunits (PubMed:10219239, PubMed:11034315, PubMed:11101505, PubMed:12185453, PubMed:20533308). KCNE2 beta subunit modulates the gating kinetics and enhances stability of the channel complex (PubMed:10219239, PubMed:11034315, PubMed:11101505, PubMed:12185453, PubMed:20533308). Alters the gating of the delayed rectifier Kv channel containing KCNB1 alpha subunit (PubMed:11101505, PubMed:20533308). Associates with KCNH2/HERG alpha subunit Kv channel to form the rapidly activating component of the delayed rectifying potassium current (IKr) in heart (PubMed:10219239, PubMed:12185453). May associate with KCNQ2 and/or KCNQ3 alpha subunits to modulate the native M-type current (PubMed:11034315). May associate with HCN1 and HCN2 channel subunits to increase potassium current (By similarity). Forms a heterooligomer complex with KCNQ1/KVLQT1 alpha subunits which leads to currents with an apparently instantaneous activation, a rapid deactivation process and a linear current-voltage relationship and decreases the amplitude of the outward current (PubMed:11101505). KCNQ1-KCNE2 channel associates with Na(+)-coupled myo-inositol symporter in the apical membrane of choroid plexus epithelium and regulates the myo-inositol gradient between blood and cerebrospinal fluid with an impact on neuron excitability (By similarity)