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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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CCP110
centriolar coiled-coil protein 110
Chromosome 16 · 16p12.3
NCBI Gene: 9738Ensembl: ENSG00000103540.18HGNC: HGNC:24342UniProt: O43303
107PubMed Papers
20Diseases
0Drugs
2Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingmicrotubule bindingcentriole replicationcentrosome duplicationneurodegenerative diseaseposterior cortical atrophyotopalatodigital syndrome type 2femoral-facial syndrome
✦AI Summary

CCP110 (centriolar coiled-coil protein 110) is a critical regulator of both centrosome duplication and ciliogenesis. The protein acts as a key negative regulator of ciliogenesis by capping the mother centriole and preventing cilia formation in collaboration with CEP97 1. At the molecular level, CCP110 autonomously binds microtubule plus ends, blocks their growth, and inhibits depolymerization, while directly interacting with CPAP to impose extremely slow processive microtubule growth essential for centriole elongation 2. During ciliogenesis, CCP110 must be removed from the mother centriole by EHD1 and MICAL-L1 to permit cilium formation 1. CCP110 also plays essential roles in spindle assembly, as it is a component of the human oocyte microtubule organizing center (huoMTOC) that regulates spindle formation during meiosis 3. Disease relevance includes potential associations with tuberculous meningitis susceptibility 4 and ciliopathies through its interactions with CEP76 5. CCP110 expression is also regulated by glucocorticoid signaling during renal development, with disrupted signaling causing ciliogenesis defects 6. These findings establish CCP110 as a fundamental component controlling centrosome-cilium biology and cellular organization.

Sources cited
1
CCP110 acts as negative regulator of ciliogenesis by capping mother centriole and must be removed by EHD1 and MICAL-L1 for cilium formation
PMID: 31615969
2
CCP110 autonomously binds microtubule plus ends, blocks growth, inhibits depolymerization, and interacts with CPAP to control slow microtubule elongation
PMID: 39847124
3
CCP110 is an essential component of the human oocyte microtubule organizing center (huoMTOC) that regulates meiotic spindle assembly
PMID: 36395215
4
CCP110 shows nominal association with tuberculous meningitis susceptibility
PMID: 35401413
5
CCP110 interacts with CEP76 and is involved in ciliopathy-related cellular dysfunction
PMID: 41105778
6
CCP110 expression is regulated by glucocorticoid signaling and is important for normal ciliogenesis in renal tubules
PMID: 40247090
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.49Moderate
posterior cortical atrophyOpen Targets
0.07Suggestive
otopalatodigital syndrome type 2Open Targets
0.04Suggestive
femoral-facial syndromeOpen Targets
0.03Suggestive
Holt-Oram syndromeOpen Targets
0.03Suggestive
breast cancerOpen Targets
0.03Suggestive
Lung agenesis - heart defect - thumb anomaliesOpen Targets
0.03Suggestive
lung agenesis-heart defect-thumb anomalies syndromeOpen Targets
0.03Suggestive
Beemer-Langer syndromeOpen Targets
0.03Suggestive
Short rib-polydactyly syndrome, Beemer-Langer typeOpen Targets
0.03Suggestive
pelvis-shoulder dysplasiaOpen Targets
0.03Suggestive
bacterial pneumoniaOpen Targets
0.03Suggestive
congenital rubellaOpen Targets
0.03Suggestive
endometrial cancerOpen Targets
0.02Suggestive
cardiomyopathyOpen Targets
0.02Suggestive
Keratoconjunctivitis siccaOpen Targets
0.02Suggestive
rhabdomyosarcomaOpen Targets
0.02Suggestive
cystOpen Targets
0.01Suggestive
dry eye syndromeOpen Targets
0.01Suggestive
ciliopathyOpen Targets
0.01Suggestive
Pathogenic Variants2
NM_001323572.2(CCP110):c.1129C>T (p.Arg377Ter)Likely pathogenic
See cases
☆☆☆☆2023→ Residue 377
NM_001323572.2(CCP110):c.856_857del (p.Val286fs)Likely pathogenic
See cases
☆☆☆☆2023→ Residue 286
View on ClinVar ↗
Related Genes
CEP104Protein interaction100%CC2D2AProtein interaction100%CALML4Protein interaction100%CALML5Protein interaction100%CALML3Protein interaction100%CALML6Protein interaction100%
Tissue Expression6 tissues
Bone Marrow
100%
Brain
83%
Ovary
30%
Heart
24%
Lung
21%
Liver
12%
Gene Interaction Network
Click a node to explore
CCP110CEP104CC2D2ACALML4CALML5CALML3CALML6
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt O43303
View on AlphaFold ↗
RankingsWhere CCP110 stands among ~20K protein-coding genes
  • #4,425of 20,598
    Most Researched107 · top quartile
  • #4,305of 5,498
    Most Pathogenic Variants2
Genes detectedCCP110
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
The mechanism of acentrosomal spindle assembly in human oocytes.
PMID: 36395215
Science · 2022
1.00
2
The landscape of genetic diseases in Saudi Arabia based on the first 1000 diagnostic panels and exomes.
PMID: 28600779
Hum Genet · 2017
0.90
3
Deciphering Genetic Susceptibility to Tuberculous Meningitis.
PMID: 35401413
Front Neurol · 2022
0.80
4
CEP76 impairment at the centrosome-cilium interface contributes to a spectrum of ciliopathies.
PMID: 41105778
Sci Adv · 2025
0.70
5
Knockdown of eIF3a alleviates pulmonary arterial hypertension by inhibiting endothelial-to-mesenchymal transition via TGFβ1/SMAD pathway.
PMID: 40346622
J Transl Med · 2025
0.60