AIRIM

Chr 1

AFG2 interacting ribosome maturation factor

Also known as: C1orf109

The protein is part of the 55LCC ATPase complex that maintains DNA replication fork progression and genome stability, and also facilitates cytoplasmic maturation of ribosomal subunits. Mutations in AIRIM cause autosomal recessive developmental delay with seizures and microcephaly, typically presenting in early infancy. The gene shows minimal constraint against loss-of-function variants (pLI <0.001), consistent with the recessive inheritance pattern observed clinically.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 1.40
Clinical SummaryAIRIM
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
1.40LOEUF
pLI 0.000
Z-score 0.57
OE 0.82 (0.511.40)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.27Z-score
OE missense 0.93 (0.791.09)
104 obs / 112.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.82 (0.511.40)
00.351.4
Missense OE0.93 (0.791.09)
00.61.4
Synonymous OE0.81
01.21.6
LoF obs/exp: 10 / 12.1Missense obs/exp: 104 / 112.2Syn Z: 1.05

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

AIRIM · 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