VARS2

Chr 6AR

valyl-tRNA synthetase 2, mitochondrial

Also known as: COXPD20, VALRS, VARS2L, VARSL

This gene encodes a mitochondrial aminoacyl-tRNA synthetase, which catalyzes the attachment of valine to tRNA(Val) for mitochondrial translation. Mutations in this gene cause combined oxidative phosphorylation deficiency-20, and are also associated with early-onset mitochondrial encephalopathies. Alternative splicing of this gene results in multiple transcript variants. [provided by RefSeq, Aug 2014]

OMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Combined oxidative phosphorylation deficiency 20MIM #615917
AR

Clinical highlights

Gene-disease validity (ClinGen)
mitochondrial disease · ARDefinitivesufficient evidence for diagnostic panels
0
Active trials
6
Pubs (1 yr)
P/LP submissions
P/LP missense
0.69
LOEUF
GOF
Mechanism· predicted

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.69LOEUF
pLI 0.000
Z-score 3.73
OE 0.53 (0.410.69)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
2.39Z-score
OE missense 0.73 (0.670.79)
451 obs / 618.4 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios?
LoF OE?0.53 (0.410.69)
00.351.4
Missense OE?0.73 (0.670.79)
00.61.4
Synonymous OE?0.84
01.21.6
LoF obs/exp: 39 / 73.6Missense obs/exp: 451 / 618.4Syn Z: 2.02

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

VARS2 · 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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