CCP110

CCP110 (Centriolar Coiled-Coil Protein 110) is a Protein Coding gene. Diseases associated with CCP110 include Spinocerebellar Ataxia 11 and Townes-Brocks Syndrome. Among its related pathways are Organelle biogenesis and maintenance and Metabolism of proteins.

Full Name

Centriolar Coiled-Coil Protein 110

Function

Necessary for centrosome duplication at different stages of procentriole formation. Acts as a key negative regulator of ciliogenesis in collaboration with CEP97 by capping the mother centriole thereby preventing cilia formation (PubMed:17719545 PubMed:17681131, PubMed:23486064).
Also involved in promoting ciliogenesis. May play a role in the assembly of the mother centriole subdistal appendages (SDA) thereby effecting the fusion of recycling endosomes to basal bodies during cilia formation (By similarity).
Required for correct spindle formation and has a role in regulating cytokinesis and genome stability via cooperation with CALM1 and CETN2 (PubMed:16760425).

Biological Process

Centriole replication Source: UniProtKB
Centrosome duplication Source: UniProtKB
Ciliary basal body organization Source: UniProtKB
Ciliary basal body-plasma membrane docking Source: Reactome
G2/M transition of mitotic cell cycle Source: Reactome
Negative regulation of centriole elongation Source: GO_Central
Negative regulation of cilium assembly Source: UniProtKB
Positive regulation of cilium assembly Source: UniProtKB
Protein deubiquitination Source: Reactome
Regulation of cytokinesis Source: UniProtKB
Regulation of G2/M transition of mitotic cell cycle Source: Reactome

Cellular Location

Centriole; Centrosome; Cilium basal body. Recruited early and then associates with the growing distal tips. Recruited to the mother centriole by KIF24. Removed from centrioles by TTBK2, leading to initiation of ciliogenesis and localizes only to the daughter centriole in ciliated cells. In cytotoxic T lymphocytes remains associated with the mother centriole during docking of the centrosome at the immunological synapse upon target contact.

PTM

Phosphorylated by CDKs.
Ubiquitinated by the SCF(CCNF) during G2 phase, leading to its degradation by the proteasome and preventing centrosome reduplication. Deubiquitinated by USP33 in S and G2/M phase, leading to stabilize CCP110 during the period which centrioles duplicate and elongate.