MARS2

Chr 2AR

methionyl-tRNA synthetase 2, mitochondrial

Also known as: COXPD25, MetRS, mtMetRS

This gene produces a mitochondrial methionyl-tRNA synthetase protein that is encoded by the nuclear genome and imported to the mitochondrion. This protein likely functions as a monomer and is predicted to localize to the mitochondrial matrix. Mutations in this gene are associated with the autosomal recessive neurodegenerative disease spastic ataxia-3 (SPAX3). [provided by RefSeq, Apr 2014]

OMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

?Combined oxidative phosphorylation deficiency 25MIM #616430
AR
Spastic ataxia 3, autosomal recessiveMIM #611390
AR

Clinical highlights

Gene-disease validity (ClinGen)
mitochondrial disease · ARDefinitivesufficient evidence for diagnostic panels
0
Active trials
14
Pubs (1 yr)
P/LP submissions
P/LP missense
0.87
LOEUF
Multiple*
Mechanism· predicted

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.87LOEUF
pLI 0.000
Z-score 1.99
OE 0.51 (0.320.87)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.30Z-score
OE missense 0.95 (0.871.05)
316 obs / 331.3 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.51 (0.320.87)
00.351.4
Missense OE?0.95 (0.871.05)
00.61.4
Synonymous OE?1.11
01.21.6
LoF obs/exp: 10 / 19.5Missense obs/exp: 316 / 331.3Syn Z: -1.02

ClinVar

No ClinVar data available.

Protein Context — Lollipop Plot

MARS2 · 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 →