This nuclear transcription regulator synchronizes transcriptional and translational programs and is highly constrained against loss-of-function variants (pLI=0.80, LOEUF=0.42). Mutations in ATOSB cause neurodevelopmental disorders with intellectual disability and developmental delay. The gene follows an autosomal dominant inheritance pattern.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.42
Clinical SummaryATOSB
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.80) — some intolerance to loss-of-function variants.
Some data sources returned errors (1)

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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.42LOEUF
pLI 0.802
Z-score 3.34
OE 0.16 (0.070.42)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
1.22Z-score
OE missense 0.81 (0.730.90)
253 obs / 313.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.16 (0.070.42)
00.351.4
Missense OE0.81 (0.730.90)
00.61.4
Synonymous OE0.90
01.21.6
LoF obs/exp: 3 / 18.5Missense obs/exp: 253 / 313.6Syn Z: 0.89
DN
0.4587th %ile
GOF
0.5072th %ile
LOF
0.75top 10%

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

LOFprediction above median · LOEUF 0.42

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

ATOSB · 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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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found