MRPL50

Chr 9

mitochondrial ribosomal protein L50

Also known as: MRP-L50, mL50

The protein encodes a component of the large 39S subunit of mitochondrial ribosomes, which synthesize proteins essential for oxidative phosphorylation within mitochondria. Pathogenic mutations in MRPL50 cause autosomal recessive mitochondrial disease characterized by severe developmental delay, epilepsy, and brain abnormalities. The low constraint scores (pLI and LOEUF) suggest this gene tolerates loss-of-function variation, consistent with recessive inheritance where heterozygous carriers are typically unaffected.

OMIMResearchSummary from RefSeq
LOEUF 1.65
Clinical SummaryMRPL50
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
1.65LOEUF
pLI 0.000
Z-score 0.23
OE 0.91 (0.511.65)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.73Z-score
OE missense 1.22 (1.041.44)
104 obs / 85.0 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.91 (0.511.65)
00.351.4
Missense OE1.22 (1.041.44)
00.61.4
Synonymous OE1.01
01.21.6
LoF obs/exp: 7 / 7.7Missense obs/exp: 104 / 85.0Syn Z: -0.05

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

MRPL50 · 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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