C18ORF21

Chr 18

ribonuclease MRP subunit p24

Also known as: C18orf21, HsT3108, PNAS-124, PNAS-131, XTP13

This protein is a specific component of the MRP ribonucleoprotein endoribonuclease complex that processes precursor ribosomal RNA. Mutations cause autosomal recessive primary microcephaly, typically presenting in infancy with severe intellectual disability and growth retardation. The gene shows low constraint against loss-of-function variants, which is consistent with the recessive inheritance pattern observed in affected families.

Summary from UniProt
Research Assistant →
0
Active trials
3
Pubs (1 yr)
4
P/LP submissions
P/LP missense
1.55
LOEUF
Mechanism
Clinical SummaryC18ORF21
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
4 unique Pathogenic / Likely Pathogenic· 2 VUS of 6 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.55LOEUF
pLI 0.000
Z-score 0.23
OE 0.92 (0.571.55)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.30Z-score
OE missense 0.92 (0.791.08)
106 obs / 115.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.92 (0.571.55)
00.351.4
Missense OE0.92 (0.791.08)
00.61.4
Synonymous OE0.79
01.21.6
LoF obs/exp: 10 / 10.8Missense obs/exp: 106 / 115.1Syn Z: 1.08

ClinVar Variant Classifications

6 submitted variants in ClinVar

Classification Summary

Pathogenic3
Likely Pathogenic1
VUS2
3
Pathogenic
1
Likely Pathogenic
2
VUS

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories· variant type breakdown unavailable

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
3
Likely Pathogenic
1
VUS
2
Likely Benign
0
Benign
0
Total6

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

C18ORF21 · 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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Key Publications
Landmark & review papers · by relevance
PubMed
Top 4 results · since 2015Search PubMed ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found