MRPL3

Chr 3AR

mitochondrial ribosomal protein L3

Also known as: COXPD9, MRL3, RPML3, uL3m

The protein is a component of the large 39S subunit of mitochondrial ribosomes and functions in mitochondrial protein synthesis. Biallelic mutations cause combined oxidative phosphorylation deficiency 9, inherited in an autosomal recessive pattern. The pathogenic mechanism involves impaired mitochondrial protein synthesis leading to defective oxidative phosphorylation.

Summary from RefSeq, OMIM, UniProt
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Primary Disease Associations & Inheritance

Combined oxidative phosphorylation deficiency 9MIM #614582
AR
0
Active trials
7
Pubs (1 yr)
0
P/LP submissions
P/LP missense
1.38
LOEUF
Mechanism
Clinical SummaryMRPL3
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Gene-Disease Validity (ClinGen)
mitochondrial disease · 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.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.38LOEUF
pLI 0.000
Z-score 0.25
OE 0.94 (0.661.38)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.16Z-score
OE missense 1.03 (0.921.16)
200 obs / 193.7 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.94 (0.661.38)
00.351.4
Missense OE1.03 (0.921.16)
00.61.4
Synonymous OE1.14
01.21.6
LoF obs/exp: 19 / 20.2Missense obs/exp: 200 / 193.7Syn Z: -0.96

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

MRPL3 · 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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Clinical Literature
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