UBN1

Chr 16

ubinuclein 1

Also known as: VT, VT4

The protein encoded by UBN1 regulates chromatin assembly and formation of senescence-associated heterochromatin foci that repress proliferation-promoting genes. Mutations cause autosomal dominant intellectual disability with microcephaly and seizures, typically presenting in early childhood. This gene is highly constrained against loss-of-function variants (pLI = 1.0, LOEUF = 0.21), indicating that haploinsufficiency is likely not tolerated.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.21
Clinical SummaryUBN1
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 1.00). One damaged copy is likely sufficient to cause disease.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.21LOEUF
pLI 1.000
Z-score 5.89
OE 0.10 (0.050.21)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
-0.79Z-score
OE missense 1.09 (1.021.16)
691 obs / 635.0 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.10 (0.050.21)
00.351.4
Missense OE1.09 (1.021.16)
00.61.4
Synonymous OE1.24
01.21.6
LoF obs/exp: 5 / 49.9Missense obs/exp: 691 / 635.0Syn Z: -3.14
DN
0.3097th %ile
GOF
0.2796th %ile
LOF
0.82top 5%

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

LOFprediction above median · LOEUF 0.21

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

UBN1 · 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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