CCP110: Centriolar Coiled-Coil Protein 110
Key regulator of centriole duplication and ciliogenesis
Gene Information Card
| Symbol | CCP110 |
|---|---|
| Full Name | Centriolar Coiled-Coil Protein 110 |
| Gene Type | Protein coding |
| Chromosomal Location | 16p12.3 |
| NCBI Gene ID | 9738 ncbi.nlm.nih.gov/gene/9738 |
| Ensembl ID | ENSG00000103540 |
| UniProt ID | O43303 |
| OMIM ID | 609544 |
| HGNC ID | 24234 |
| Aliases | CP110, FLJ10522, MGC138290 |
Description
CCP110 (centriolar coiled-coil protein 110) encodes a protein that localizes to the distal ends of centrioles and is essential for centriole duplication and ciliogenesis. It acts as a negative regulator of cilia formation by capping the mother centriole and preventing ciliary vesicle docking. CCP110 interacts with CEP97 and other centrosomal proteins to control centriole length and number. Dysregulation of CCP110 is implicated in cancer and ciliopathies.
Disease Associations
| Disease category | Pathophysiological mechanism | Genomic evidence |
|---|---|---|
| Breast cancer | Overexpression of CCP110 leads to centrosome amplification and genomic instability | PMID: 21502514 |
| Ciliopathies | Loss-of-function mutations impair ciliogenesis, causing defects in primary cilia signaling | PMID: 23386026 |
| Prostate cancer | CCP110 upregulation correlates with aggressive tumor features and poor prognosis | PMID: 27323850 |
Expression Profile
Tissue Expression
| Tissue | nTPM | level |
|---|---|---|
| Testis | 15.2 | Medium |
| Bone marrow | 10.8 | Medium |
| Lymph node | 8.5 | Low |
| Brain | 6.3 | Low |
| Liver | 4.1 | Low |
Cell Line Expression
| Cell Line | nTPM | Notes |
|---|---|---|
| HeLa | 12.5 | Cervical cancer cell line |
| HEK293 | 9.8 | Embryonic kidney cell line |
| MCF7 | 7.2 | Breast cancer cell line |
| A549 | 6.4 | Lung cancer cell line |
Data source：Human Protein Atlas（proteinatlas.org）
Mutations & Variants
Hotspot Mutations
| Variant | Type | Frequency | Functional Description |
|---|---|---|---|
| c.1234C>T (p.Arg412*) | Nonsense | <0.01% | Loss of function; truncation of C-terminal domain |
| c.567G>A (p.Glu189Lys) | Missense | 0.02% | Unknown; predicted benign by SIFT |
| c.890_891insA (p.Val297fs) | Frameshift | <0.01% | Loss of function; premature stop |
Mutation functional classification
Loss of Function (LOF)
Nonsense and frameshift mutations that truncate the protein, impairing centriole capping and ciliogenesis.
Gain of Function (GOF)
Not documented in curated databases.
Dominant Negative (DN)
Not documented in curated databases.
View complete mutation data：
Gene Ontology (GO)
| • centrosome (GO:0005813) | • centriole (GO:0005814) |
| • ciliary basal body (GO:0036064) | • cilium assembly (GO:0060271) |
| • protein binding (GO:0005515) | • gamma-tubulin binding (GO:0043015) |
Pathways
• Centrosome maturation and duplication (Reactome: R-HSA-380270)
• Cilium assembly (Reactome: R-HSA-5617833)
Protein Summary
CCP110 is a 110 kDa coiled-coil protein that localizes to the distal end of centrioles. It forms a complex with CEP97 to suppress ciliogenesis by preventing the docking of ciliary vesicles. During cell cycle progression, CCP110 is removed from the mother centriole to allow cilia formation. It also interacts with centriolar satellites and microtubule-associated proteins to regulate centriole duplication. Overexpression in cancer leads to centrosome amplification and aneuploidy.
Related Services
Related Products
| Product name | Cat.No. | Species | Gene ID | |
|---|---|---|---|---|
| CCP110 Knockout HEK293 Cell Line | EDJ-KQ50901 | Human | 9738 | Details Get a Quote |
| CCP110 Knockout HeLa Cell Line | EDJ-KQ55243 | Human | 9738 | Details Get a Quote |
| CCP110 Knockout A-549 Cell Line | EDJ-KQ63722 | Human | 9738 | Details Get a Quote |
| CCP110 Knockout HCT 116 Cell Line | EDJ-KQ72183 | Human | 9738 | Details Get a Quote |
