This gene encodes a member of the homeobox transcription factor family. A highly related protein in mouse has been shown to influence cellular processes that control cell adhesion and remodeling of the actin cytoskeleton in myoblast fusion and chondrogenesis. The encoded protein may also play a role in cancer progression. [provided by RefSeq, Jul 2008]

0
Active trials
4
Pubs (1 yr)
75
P/LP submissions
0%
P/LP missense
0.69
LOEUF
DN
Mechanism· predicted
Clinical SummaryBARX2
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.27) despite low pLI — interpret in context.
📋
ClinVar Variants
75 unique Pathogenic / Likely Pathogenic· 38 VUS of 123 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.69LOEUF
pLI 0.212
Z-score 2.29
OE 0.27 (0.120.69)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
-0.09Z-score
OE missense 1.02 (0.901.16)
171 obs / 167.7 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.27 (0.120.69)
00.351.4
Missense OE1.02 (0.901.16)
00.61.4
Synonymous OE0.94
01.21.6
LoF obs/exp: 3 / 11.3Missense obs/exp: 171 / 167.7Syn Z: 0.41
DN
0.6162th %ile
GOF
0.4973th %ile
LOF
0.49top 25%

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

123 submitted variants in ClinVar

Classification Summary

Pathogenic71
Likely Pathogenic4
VUS38
Likely Benign2
Benign2
71
Pathogenic
4
Likely Pathogenic
38
VUS
2
Likely Benign
2
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
71
0
71
Likely Pathogenic
0
0
4
0
4
VUS
0
36
2
0
38
Likely Benign
0
2
0
0
2
Benign
0
1
0
1
2
Total039771117

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

View in ClinVar →

Protein Context — Lollipop Plot

BARX2 · protein map & ClinVar variants

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

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →