C9ORF40

Chr 9

chromosome 9 open reading frame 40

C9ORF40 encodes a protein involved in ribosome biogenesis and RNA processing. Mutations cause autosomal recessive neurodevelopmental disorders characterized by intellectual disability, developmental delay, and neurological abnormalities. This gene shows relatively low constraint against loss-of-function variants, consistent with its recessive inheritance pattern.

LOEUF 1.49
Clinical SummaryC9ORF40
Population Constraint (gnomAD)
Low constraint (pLI 0.07) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
38 unique Pathogenic / Likely Pathogenic· 7 VUS of 47 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.49LOEUF
pLI 0.070
Z-score 0.93
OE 0.50 (0.201.49)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
1.00Z-score
OE missense 0.69 (0.560.86)
58 obs / 83.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.50 (0.201.49)
00.351.4
Missense OE0.69 (0.560.86)
00.61.4
Synonymous OE0.70
01.21.6
LoF obs/exp: 2 / 4.0Missense obs/exp: 58 / 83.9Syn Z: 1.41

ClinVar Variant Classifications

47 submitted variants in ClinVar

Classification Summary

Pathogenic34
Likely Pathogenic4
VUS7
34
Pathogenic
4
Likely Pathogenic
7
VUS

Curated Variants Distribution

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

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
34
Likely Pathogenic
4
VUS
7
Likely Benign
0
Benign
0
Total45

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

C9ORF40 · 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.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 1 results · since 2015Search PubMed ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found