C9ORF152

Chr 9

chromosome 9 open reading frame 152

Also known as: bA470J20.2

This gene encodes a protein of unknown function. Mutations cause autosomal recessive developmental and epileptic encephalopathy with movement abnormalities, characterized by early-onset seizures, developmental delay, and dystonia. The gene shows minimal constraint against loss-of-function variants, consistent with the recessive inheritance pattern observed in affected patients.

LOEUF 1.05
Clinical SummaryC9ORF152
Population Constraint (gnomAD)
Low constraint (pLI 0.02) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
34 unique Pathogenic / Likely Pathogenic· 1 VUS of 42 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.05LOEUF
pLI 0.018
Z-score 1.48
OE 0.46 (0.231.05)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.65Z-score
OE missense 1.16 (1.021.33)
147 obs / 126.5 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.46 (0.231.05)
00.351.4
Missense OE1.16 (1.021.33)
00.61.4
Synonymous OE0.98
01.21.6
LoF obs/exp: 4 / 8.7Missense obs/exp: 147 / 126.5Syn Z: 0.12

ClinVar Variant Classifications

42 submitted variants in ClinVar

Classification Summary

Pathogenic29
Likely Pathogenic5
VUS1
Likely Benign3
29
Pathogenic
5
Likely Pathogenic
1
VUS
3
Likely Benign

Curated Variants Distribution

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

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
29
Likely Pathogenic
5
VUS
1
Likely Benign
3
Benign
0
Total38

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

C9ORF152 · 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