BARX1 encodes a transcription factor that regulates craniofacial development, tooth formation, and stomach organogenesis. Mutations cause autosomal dominant non-syndromic tooth agenesis, particularly affecting molar development. The gene shows moderate constraint against loss-of-function variants, consistent with its role in developmental processes.

OMIMResearchSummary from UniProt
LOFmechanismLOEUF 0.68
Clinical SummaryBARX1
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.59) — some intolerance to loss-of-function variants.
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ClinVar Variants
32 unique Pathogenic / Likely Pathogenic· 37 VUS of 71 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.68LOEUF
pLI 0.586
Z-score 2.09
OE 0.14 (0.050.68)
Moderately constrained

Typical tolerance to LoF variation

Missense Constraint
1.07Z-score
OE missense 0.71 (0.590.86)
75 obs / 105.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.14 (0.050.68)
00.351.4
Missense OE0.71 (0.590.86)
00.61.4
Synonymous OE0.97
01.21.6
LoF obs/exp: 1 / 6.9Missense obs/exp: 75 / 105.9Syn Z: 0.17
DN
0.5180th %ile
GOF
0.3987th %ile
LOF
0.72top 10%

The highest-scoring mechanism for this gene is loss-of-function (haploinsufficiency).

LOFprediction above median

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

ClinVar Variant Classifications

71 submitted variants in ClinVar

Classification Summary

Pathogenic28
Likely Pathogenic4
VUS37
28
Pathogenic
4
Likely Pathogenic
37
VUS

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
28
0
28
Likely Pathogenic
0
0
4
0
4
VUS
0
35
2
0
37
Likely Benign
0
0
0
0
0
Benign
0
0
0
0
0
Total03534069

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

BARX1 · 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.

Search ClinicalTrials.gov →
Clinical Literature
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Full-Text Mentions
NLP-detected gene mentions in article bodies · via PubTator3
PubTator3
Top 2 full-text resultsSearch PubTator3 ↗