GJA9

Chr 1

gap junction protein alpha 9

Also known as: CX58, CX59, GJA10

GJA9 encodes connexin-59, a protein that forms gap junction channels allowing direct intercellular communication through the passage of small molecules between adjacent cells. Mutations cause oculodentodigital dysplasia, an autosomal dominant disorder affecting the eyes, teeth, and digits. The gene shows minimal constraint against loss-of-function variants, suggesting tolerance to reduced protein levels.

OMIMResearchSummary from RefSeq, UniProt
MultiplemechanismLOEUF 1.56
Clinical SummaryGJA9
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
1.56LOEUF
pLI 0.000
Z-score -0.17
OE 1.05 (0.711.56)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.94Z-score
OE missense 0.84 (0.750.94)
222 obs / 264.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE1.05 (0.711.56)
00.351.4
Missense OE0.84 (0.750.94)
00.61.4
Synonymous OE0.87
01.21.6
LoF obs/exp: 17 / 16.3Missense obs/exp: 222 / 264.8Syn Z: 1.03
DN
0.77top 25%
GOF
0.83top 5%
LOF
0.3746th %ile

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

GOFprediction above median
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

GJA9 · 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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