CCDC9

Chr 19

coiled-coil domain containing 9

CCDC9 encodes a component of the exon junction complex that binds RNA and directs post-transcriptional processes including mRNA export, nonsense-mediated decay, and translation. Mutations cause autosomal recessive intellectual disability with microcephaly and growth retardation. The gene is highly intolerant to loss-of-function variants, suggesting complete loss of protein function may be embryonic lethal.

ResearchSummary from RefSeq, UniProt
DNmechanismLOEUF 0.73
Clinical SummaryCCDC9
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
0.73LOEUF
pLI 0.000
Z-score 2.62
OE 0.45 (0.290.73)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
-0.18Z-score
OE missense 1.03 (0.941.12)
344 obs / 334.8 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.45 (0.290.73)
00.351.4
Missense OE1.03 (0.941.12)
00.61.4
Synonymous OE1.16
01.21.6
LoF obs/exp: 12 / 26.5Missense obs/exp: 344 / 334.8Syn Z: -1.42
DN
0.6162th %ile
GOF
0.5759th %ile
LOF
0.4331th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

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

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

CCDC9 · 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
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