KCTD7

Chr 7AR

potassium channel tetramerization domain containing 7

Also known as: CLN14, EPM3

This gene encodes a member of the potassium channel tetramerization domain-containing protein family that may control excitability of cortical neurons. Mutations cause progressive myoclonic epilepsy-3 with or without intracellular inclusions, inherited in an autosomal recessive pattern. The gene shows moderate constraint against loss-of-function variants (LOEUF 0.654), consistent with its role in neuronal excitability control.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 0.651 OMIM phenotype
Clinical SummaryKCTD7
🧬
Gene-Disease Validity (ClinGen)
progressive myoclonus epilepsy · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
💊
Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.65LOEUF
pLI 0.001
Z-score 2.90
OE 0.39 (0.240.65)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.47Z-score
OE missense 0.75 (0.670.84)
210 obs / 279.0 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.39 (0.240.65)
00.351.4
Missense OE0.75 (0.670.84)
00.61.4
Synonymous OE0.90
01.21.6
LoF obs/exp: 10 / 25.9Missense obs/exp: 210 / 279.0Syn Z: 0.75
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveKCTD7-related progressive myoclonic epilepsyLOFAR

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.6936th %ile
GOF
0.5856th %ile
LOF
0.2776th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

KCTD7 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold
Clinical Literature
Open Research Assistant →