AARS2

Chr 6

alanyl-tRNA synthetase 2, mitochondrial

Also known as: AARSL, COXPD8, LKENP, MT-ALARS, MTALARS

The protein encoded by this gene belongs to the class-II aminoacyl-tRNA synthetase family. Aminoacyl-tRNA synthetases play critical roles in mRNA translation by charging tRNAs with their cognate amino acids. The encoded protein is a mitochondrial enzyme that specifically aminoacylates alanyl-tRNA. Mutations in this gene are a cause of combined oxidative phosphorylation deficiency 8. [provided by RefSeq, Dec 2011]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtCombined oxidative phosphorylation deficiency 8
UniProtLeukoencephalopathy, progressive, with ovarian failure

Clinical highlights

Gene-disease validity (ClinGen)
mitochondrial disease · ARDefinitivesufficient evidence for diagnostic panels
0
Active trials
31
Pubs (1 yr)
P/LP submissions
P/LP missense
0.83
LOEUF
Multiple*
Mechanism· predicted
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GeneReview available — AARS2
Authoritative clinical overview · Recommended first read
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Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.83LOEUF
pLI 0.000
Z-score 2.56
OE 0.63 (0.480.83)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.07Z-score
OE missense 0.99 (0.931.06)
567 obs / 571.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.63 (0.480.83)
00.351.4
Missense OE?0.99 (0.931.06)
00.61.4
Synonymous OE?0.84
01.21.6
LoF obs/exp: 35 / 55.6Missense obs/exp: 567 / 571.9Syn Z: 1.88

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

AARS2 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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