CEP152

Description

The CEP152 (centrosomal protein 152) is a protein-coding gene located on chromosome 15.

CEP152 is a protein essential for centriole duplication. It is the human ortholog of the Drosophila melanogaster gene asterless (asl). CEP152 is involved in centrosome duplication, and its function seems also to involve CEP63, CDK5RAP2 and WDR62 through a stepwise assembled complex at the centrosome that recruits CDK2 required for centriole duplication. It acts as a molecular scaffold facilitating the interaction of PLK4 and CENPJ, 2 molecules involved in centriole formation. CEP152 is proposed to snatch PLK4 away from PLK4:CEP92 complexes in early G1 daughter centriole and to reposition PLK4 at the outer boundary of a newly forming CEP152 ring structure. It also plays a key role in deuterosome-mediated centriole amplification in multiciliated cells that can generate more than 100 centrioles. Overexpression of CEP152 can drive amplification of centrioles.

CEP152 is essential for centrosome duplication, and its function involves the interaction with other proteins like CEP63, CDK5RAP2 and WDR62. These proteins assemble into a complex at the centrosome, which recruits CDK2, crucial for centriole duplication. CEP152 acts as a scaffold, facilitating the interaction of PLK4 and CENPJ, two molecules involved in centriole formation. CEP152 is also proposed to redirect PLK4 from its complex with CEP92 to a newly forming CEP152 ring structure. It plays a critical role in deuterosome-mediated centriole amplification, particularly in multiciliated cells where over 100 centrioles can be generated. Overexpression of CEP152 can trigger centriole amplification.

CEP152 is also known as MCPH4, MCPH9, SCKL5.

Associated Diseases

• Microcephaly 9, primary, autosomal recessive
• Seckel syndrome 5
• Autosomal recessive primary microcephaly
• Seckel syndrome