The ATRIP protein is essential for DNA damage checkpoint signaling and is required for ATR protein expression and stabilization. Mutations cause Seckel syndrome, characterized by severe microcephaly, intrauterine and postnatal growth restriction, and distinctive facial features, with an autosomal recessive inheritance pattern. This gene is highly constrained against loss-of-function variants, reflecting its critical cellular role.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.64
Clinical SummaryATRIP
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Gene-Disease Validity (ClinGen)
hereditary breast carcinoma · ADLimited

Limited evidence — not for standalone diagnostic reporting

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.64LOEUF
pLI 0.000
Z-score 3.22
OE 0.41 (0.270.64)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.05Z-score
OE missense 0.86 (0.780.93)
361 obs / 422.0 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.41 (0.270.64)
00.351.4
Missense OE0.86 (0.780.93)
00.61.4
Synonymous OE0.90
01.21.6
LoF obs/exp: 14 / 34.3Missense obs/exp: 361 / 422.0Syn Z: 1.09

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ATRIP · 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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