AIMP1

Chr 4AR

aminoacyl tRNA synthetase complex interacting multifunctional protein 1

Also known as: EMAP2, EMAPII, HLD3, SCYE1, p43

The protein functions as the p43 subunit of the multi-tRNA synthetase complex, modulating aminoacylation activity of tRNA synthetase, and also acts as a cytokine involved in controlling angiogenesis, inflammation, and wound healing when released from cells. Mutations cause autosomal recessive hypomyelinating leukodystrophy type 3. The pathogenic mechanism likely involves disruption of protein synthesis through impaired tRNA aminoacylation.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 1.221 OMIM phenotype
Clinical SummaryAIMP1
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Gene-Disease Validity (ClinGen)
hypomyelinating leukodystrophy 3 · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

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
1.22LOEUF
pLI 0.000
Z-score 0.92
OE 0.75 (0.481.22)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.21Z-score
OE missense 1.04 (0.931.18)
182 obs / 174.3 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.75 (0.481.22)
00.351.4
Missense OE1.04 (0.931.18)
00.61.4
Synonymous OE0.92
01.21.6
LoF obs/exp: 12 / 16.0Missense obs/exp: 182 / 174.3Syn Z: 0.50

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

AIMP1 · 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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