C9ORF78

Chr 9

chromosome 9 open reading frame 78

Also known as: CSU2, HCA59, HSPC220, TLS1, bA409K20.3

The C9ORF78 protein binds U5 snRNA and regulates pre-mRNA splicing by promoting upstream 3'-splice site usage at alternative NAGNAG sites and modulating spliceosomal remodeling through interactions with SNRNP200, while also being required for proper chromosome segregation. The gene shows tolerance to loss-of-function variants (pLI = 0.000019, LOEUF = 1.004), and no human disease associations have been established for C9ORF78 mutations.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 1.00
Clinical SummaryC9ORF78
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
1.00LOEUF
pLI 0.000
Z-score 1.55
OE 0.59 (0.361.00)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
1.82Z-score
OE missense 0.61 (0.520.72)
105 obs / 172.3 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.59 (0.361.00)
00.351.4
Missense OE0.61 (0.520.72)
00.61.4
Synonymous OE1.04
01.21.6
LoF obs/exp: 10 / 16.9Missense obs/exp: 105 / 172.3Syn Z: -0.25

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

C9ORF78 · 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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Clinical Literature
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 1 results · since 2015Search PubMed ↗