KCNE1

Chr 21ARAD

potassium voltage-gated channel subfamily E regulatory subunit 1

Also known as: ISK, JLNS, JLNS2, LQT2/5, LQT5, MinK

The product of this gene belongs to the potassium channel KCNE family. Potassium ion channels are essential to many cellular functions and show a high degree of diversity, varying in their electrophysiologic and pharmacologic properties. This gene encodes a transmembrane protein known to associate with the product of the KVLQT1 gene to form the delayed rectifier potassium channel. Mutation in this gene are associated with both Jervell and Lange-Nielsen and Romano-Ward forms of long-QT syndrome. Alternatively spliced transcript variants encoding the same protein have been identified. [provided by RefSeq, Jul 2008]

GeneReviewsOMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Jervell and Lange-Nielsen syndrome 2MIM #612347
AR
Long QT syndrome 5MIM #613695
AD

Clinical highlights

Gene-disease validity (ClinGen)
long QT syndrome 5 · ADLimitednot for standalone diagnostic reporting
0
Active trials
56
Pubs (1 yr)
P/LP submissions
P/LP missense
1.93
LOEUF
Multiple*
Mechanism· predicted
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GeneReview available — KCNE1
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.93LOEUF
pLI 0.003
Z-score -0.63
OE 1.48 (0.561.93)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-0.62Z-score
OE missense 1.19 (1.011.41)
97 obs / 81.4 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?1.48 (0.561.93)
00.351.4
Missense OE?1.19 (1.011.41)
00.61.4
Synonymous OE?1.29
01.21.6
LoF obs/exp: 3 / 2.0Missense obs/exp: 97 / 81.4Syn Z: -1.39

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

KCNE1 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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