PARS2

Chr 1

prolyl-tRNA synthetase 2, mitochondrial

Also known as: DEE75, EIEE75, MT-PRORS, proRS

This gene encodes a putative member of the class II 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 proline to tRNA molecules. Mutations have been found in this gene in some patients with Alpers syndrome. [provided by RefSeq, Mar 2015]

ResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtDevelopmental and epileptic encephalopathy 75

Clinical highlights

Gene-disease validity (ClinGen)
mitochondrial disease · ARModerateconsider for supplementary testing
0
Active trials
4
Pubs (1 yr)
P/LP submissions
P/LP missense
0.88
LOEUF
DN
Mechanism· predicted
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?
0.88LOEUF
pLI 0.004
Z-score 1.88
OE 0.45 (0.240.88)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.29Z-score
OE missense 0.95 (0.861.05)
266 obs / 279.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.45 (0.240.88)
00.351.4
Missense OE?0.95 (0.861.05)
00.61.4
Synonymous OE?0.91
01.21.6
LoF obs/exp: 6 / 13.4Missense obs/exp: 266 / 279.5Syn Z: 0.78

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

PARS2 · 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 →