CCDC120

Chr X

coiled-coil domain containing 120

Also known as: JM11

The protein is required for centriole subdistal appendage assembly and microtubule anchoring, and promotes neurite growth by localizing CYTH2 to vesicles for transport and ARF6 activation. Mutations cause autosomal recessive intellectual disability with microcephaly, short stature, and facial dysmorphism. The gene shows moderate constraint against loss-of-function variants (LOEUF 0.55), suggesting haploinsufficiency sensitivity despite the recessive inheritance pattern.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.55
Clinical SummaryCCDC120
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.21) despite low pLI — interpret in context.
📋
ClinVar Variants
62 unique Pathogenic / Likely Pathogenic· 105 VUS of 210 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.55LOEUF
pLI 0.434
Z-score 2.74
OE 0.21 (0.100.55)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.07Z-score
OE missense 0.99 (0.891.09)
277 obs / 280.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.21 (0.100.55)
00.351.4
Missense OE0.99 (0.891.09)
00.61.4
Synonymous OE1.02
01.21.6
LoF obs/exp: 3 / 14.1Missense obs/exp: 277 / 280.5Syn Z: -0.21

ClinVar Variant Classifications

210 submitted variants in ClinVar

Classification Summary

Pathogenic60
Likely Pathogenic2
VUS105
Likely Benign6
Conflicting1
60
Pathogenic
2
Likely Pathogenic
105
VUS
6
Likely Benign
1
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
60
0
60
Likely Pathogenic
0
0
2
0
2
VUS
0
99
6
0
105
Likely Benign
0
4
0
2
6
Benign
0
0
0
0
0
Conflicting
1
Total0103682174

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

CCDC120 · 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 ↗