KCTD18

Chr 2

potassium channel tetramerization domain containing 18

The KCTD18 protein is predicted to enable identical protein binding and participate in ion transport across cell membranes and protein complex formation. Mutations cause neurodevelopmental disorders with intellectual disability and seizures, inherited in an autosomal recessive pattern. This gene shows minimal constraint against loss-of-function variants, consistent with recessive inheritance where both copies must be affected to cause disease.

ResearchSummary from RefSeq
DNmechanismLOEUF 1.42
Clinical SummaryKCTD18
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
Some data sources returned errors (1)

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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.42LOEUF
pLI 0.000
Z-score 0.41
OE 0.88 (0.561.42)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.37Z-score
OE missense 0.94 (0.841.04)
251 obs / 268.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.88 (0.561.42)
00.351.4
Missense OE0.94 (0.841.04)
00.61.4
Synonymous OE1.00
01.21.6
LoF obs/exp: 12 / 13.6Missense obs/exp: 251 / 268.1Syn Z: 0.00
DN
0.6161th %ile
GOF
0.5661th %ile
LOF
0.4529th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

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

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

KCTD18 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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