USO1

Chr 4

USO1 vesicle transport factor

The USO1 protein is a vesicular transport factor that regulates intercisternal transport in the Golgi apparatus and promotes targeting of COPII vesicles from the endoplasmic reticulum to the Golgi by forming SNARE complexes. Mutations cause autosomal recessive developmental delay with dysmorphic facies and dental anomalies. This gene is highly constrained against loss-of-function variants (LOEUF = 0.369), suggesting intolerance to complete protein loss.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.37
Clinical SummaryUSO1
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.23) despite low pLI — interpret in context.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.37LOEUF
pLI 0.334
Z-score 5.19
OE 0.23 (0.140.37)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
1.28Z-score
OE missense 0.83 (0.760.90)
367 obs / 442.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.23 (0.140.37)
00.351.4
Missense OE0.83 (0.760.90)
00.61.4
Synonymous OE0.91
01.21.6
LoF obs/exp: 12 / 52.6Missense obs/exp: 367 / 442.8Syn Z: 0.89

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

USO1 · 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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