MRPS34

Chr 16AR

mitochondrial ribosomal protein S34

This protein is required for mitochondrial translation and maintains the stability of the small ribosomal subunit and 12S rRNA needed for mitoribosome formation. Mutations cause combined oxidative phosphorylation deficiency 32, inherited in an autosomal recessive pattern. The gene shows relatively low constraint to loss-of-function variation (pLI 0.003, LOEUF 1.72).

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 1.721 OMIM phenotype
Clinical SummaryMRPS34
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Gene-Disease Validity (ClinGen)
Leigh syndrome · ARModerate

Moderate evidence — consider for supplementary testing

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
Some data sources returned errors (1)

ncbi: Error: NCBI fetch failed: 429 https://eutils.ncbi.nlm.nih.gov/entrez/eutils/esearch.fcgi

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.72LOEUF
pLI 0.003
Z-score 0.33
OE 0.84 (0.411.72)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-1.43Z-score
OE missense 1.41 (1.231.63)
135 obs / 95.7 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.84 (0.411.72)
00.351.4
Missense OE1.41 (1.231.63)
00.61.4
Synonymous OE1.35
01.21.6
LoF obs/exp: 4 / 4.8Missense obs/exp: 135 / 95.7Syn Z: -1.76
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
strongMRPS34-related Leigh syndrome with instability of the small mitoribosomal subunitLOFAR

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.4884th %ile
GOF
0.77top 25%
LOF
0.3549th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

MRPS34 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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Clinical Literature
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