BARHL1

Chr 9

BarH like homeobox 1

BARHL1 encodes a transcription factor that binds double-stranded DNA and regulates RNA polymerase II transcription, with predicted roles in nervous system development and auditory function. Mutations in BARHL1 have not been definitively linked to a specific Mendelian disorder in the provided information. The low pLI score (0.15) and moderate LOEUF score (0.77) suggest the gene has moderate tolerance to loss-of-function variants.

Summary from RefSeq
0
Active trials
1
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.77
LOEUF
DN
Mechanism· predicted
Clinical SummaryBARHL1
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.30) despite low pLI — interpret in context.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.77LOEUF
pLI 0.146
Z-score 2.07
OE 0.30 (0.130.77)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.09Z-score
OE missense 0.78 (0.680.89)
151 obs / 193.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.30 (0.130.77)
00.351.4
Missense OE0.78 (0.680.89)
00.61.4
Synonymous OE1.03
01.21.6
LoF obs/exp: 3 / 10.1Missense obs/exp: 151 / 193.6Syn Z: -0.26
DN
0.6453th %ile
GOF
0.3590th %ile
LOF
0.60top 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

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

BARHL1 · 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.

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Clinical Literature
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