CHRNB3

Chr 8

cholinergic receptor nicotinic beta 3 subunit

The CHRNB3 protein is a beta subunit component of neuronal nicotinic acetylcholine receptors, which are pentameric ligand-gated cation channels that mediate excitatory synaptic transmission in the nervous system. Mutations in CHRNB3 cause autosomal dominant nocturnal frontal lobe epilepsy, a focal epilepsy syndrome with seizures that typically occur during sleep and begin in childhood or adolescence. The gene shows low constraint against loss-of-function variants (pLI <0.001), suggesting that complete loss of function may be tolerated.

OMIMResearchSummary from RefSeq, UniProt
MultiplemechanismLOEUF 0.95
Clinical SummaryCHRNB3
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.95LOEUF
pLI 0.000
Z-score 1.71
OE 0.58 (0.360.95)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.84Z-score
OE missense 0.85 (0.760.95)
220 obs / 258.0 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.58 (0.360.95)
00.351.4
Missense OE0.85 (0.760.95)
00.61.4
Synonymous OE0.90
01.21.6
LoF obs/exp: 11 / 19.1Missense obs/exp: 220 / 258.0Syn Z: 0.84
DN
0.83top 10%
GOF
0.79top 25%
LOF
0.1895th %ile

This gene has evidence for multiple mechanisms of pathogenicity (dominant-negative and gain-of-function). Both the Badonyi & Marsh prediction and the broader genomic evidence point to dominant-negative as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

DNprediction above median
GOFprediction 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

CHRNB3 · 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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Clinical Literature
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