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Centriolar coiled-coil protein of 110 kDa (CP110)
- Target
- CP110
- Molecular classification
- Other (Centrosomal/centriolar structural protein)
01
Overview
Centriolar coiled-coil protein of 110 kDa (CP110) is a centrosomal protein encoded by the CCP110 gene in humans that localizes to the distal end of centrioles. It is a substrate for cyclin-dependent kinases and is essential for proper regulation of centriole duplication and the cell cycle. CP110 acts as a critical negative regulator of primary cilia assembly by capping the distal end of centrioles, thereby preventing unnecessary cilia formation in non-ciliated cells. Removal or loss of CP110 is necessary for cilia biogenesis and is involved in early steps such as subdistal appendage assembly and vesicle docking, though it may also play context-dependent roles as a positive regulator of ciliogenesis in vivo. Disruption of CP110 function can result in ciliopathies and is associated with disorders such as spinocerebellar ataxia 11. There are currently no known drugs that directly target this protein.
- Other names
- CCP110CEP110KIAA0419centriolar coiled-coil protein 110centriolar coiled-coil protein 110 kDacentriolar coiled coil protein 110kDacentrosomal protein CP110centrosomal protein of 110 kDacentrosomal protein of 110kDa
02
Biological functions
Regulation of centriole duplicationNegative regulation of cilium (cilia) assembly/ciliogenesisRegulation of cytokinesisPositive regulation of cilia biogenesis in certain tissue contextsAssembly of mother centriole subdistal appendagesGenome stability
03
Disease associations
Ciliopathies (e.g., short rib-polydactyly syndrome-like phenotypes in animal models)Spinocerebellar ataxia 11Female-restricted syndromic X-linked intellectual disability 99
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