FUBP3

Chr 9

far upstream element binding protein 3

Also known as: FBP3

FUBP3 encodes a transcriptional activator that binds single-stranded DNA and regulates gene expression through RNA polymerase II-mediated transcription. Mutations cause autosomal recessive intellectual disability with childhood onset, often accompanied by seizures and developmental delays. The gene shows high constraint against loss-of-function variants (LOEUF 0.364), indicating that complete loss of function is poorly tolerated.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.36
Clinical SummaryFUBP3
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.80) — some intolerance to loss-of-function variants.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.36LOEUF
pLI 0.798
Z-score 4.49
OE 0.19 (0.110.36)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
2.17Z-score
OE missense 0.67 (0.590.74)
222 obs / 333.8 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.19 (0.110.36)
00.351.4
Missense OE0.67 (0.590.74)
00.61.4
Synonymous OE0.90
01.21.6
LoF obs/exp: 7 / 36.1Missense obs/exp: 222 / 333.8Syn Z: 0.88
DN
0.5181th %ile
GOF
0.3888th %ile
LOF
0.68top 25%

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

LOFprediction above median · LOEUF 0.36

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

FUBP3 · 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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