RPL23

Chr 17

ribosomal protein L23

Also known as: L23, rpL17, uL14

The protein is a component of the large 60S ribosomal subunit that catalyzes protein synthesis in the cytoplasm. Mutations in RPL23 cause disease through loss of function, though the low pLI score (0.33) and moderate LOEUF score (0.74) suggest the gene is relatively tolerant to haploinsufficiency. Based on the available data, the specific disease phenotype and inheritance pattern associated with RPL23 mutations are not definitively established.

Summary from RefSeq, UniProt, Mechanism
0
Active trials
27
Pubs (1 yr)
7
P/LP submissions
0%
P/LP missense
0.74
LOEUF
LOF
Mechanism· predicted
Clinical SummaryRPL23
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.23) despite low pLI — interpret in context.
📋
ClinVar Variants
7 unique Pathogenic / Likely Pathogenic· 22 VUS of 76 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.74LOEUF
pLI 0.330
Z-score 2.07
OE 0.23 (0.100.74)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.95Z-score
OE missense 0.40 (0.300.53)
33 obs / 82.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.23 (0.100.74)
00.351.4
Missense OE0.40 (0.300.53)
00.61.4
Synonymous OE0.88
01.21.6
LoF obs/exp: 2 / 8.5Missense obs/exp: 33 / 82.9Syn Z: 0.53
DN
0.4388th %ile
GOF
0.2497th %ile
LOF
0.67top 25%

The highest-scoring mechanism for this gene is loss-of-function (haploinsufficiency).

LOFprediction above median

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

ClinVar Variant Classifications

76 submitted variants in ClinVar

Classification Summary

Pathogenic7
VUS22
Likely Benign35
Benign2
Conflicting1
7
Pathogenic
22
VUS
35
Likely Benign
2
Benign
1
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
7
0
7
Likely Pathogenic
0
0
0
0
0
VUS
0
13
8
1
22
Likely Benign
0
0
22
13
35
Benign
0
0
2
0
2
Conflicting
1
Total013391467

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

RPL23 · 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 →
Clinical Literature
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