EARS2

Chr 16AR

glutamyl-tRNA synthetase 2, mitochondrial

Also known as: COXPD12, MSE1, gluRS, mtGlnRS, mtGluRS

This gene encodes a member of the class I family of aminoacyl-tRNA synthetases. These enzymes play a critical role in protein biosynthesis by charging tRNAs with their cognate amino acids. This protein is encoded by the nuclear genome but is likely to be imported to the mitochondrion where it is thought to catalyze the ligation of glutamate to tRNA molecules. Mutations in this gene have been associated with combined oxidative phosphorylation deficiency 12 (COXPD12). Alternative splicing results in multiple transcript variants. [provided by RefSeq, Mar 2015]

OMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Combined oxidative phosphorylation deficiency 12MIM #614924
AR

Clinical highlights

Gene-disease validity (ClinGen)
Leigh syndrome · ARDefinitivesufficient evidence for diagnostic panels2 gene-disease associations curated in total
0
Active trials
9
Pubs (1 yr)
P/LP submissions
P/LP missense
1.02
LOEUF
DN
Mechanism· predicted

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.02LOEUF
pLI 0.000
Z-score 1.48
OE 0.67 (0.451.02)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.11Z-score
OE missense 0.98 (0.891.08)
303 obs / 308.3 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.67 (0.451.02)
00.351.4
Missense OE?0.98 (0.891.08)
00.61.4
Synonymous OE?1.05
01.21.6
LoF obs/exp: 16 / 23.8Missense obs/exp: 303 / 308.3Syn Z: -0.45

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

EARS2 · 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.

Search ClinicalTrials.gov →