Centrosomal protein 57 (CEP57)

Centrosomal protein 57 (CEP57) is a highly conserved coiled-coil scaffold protein localized at the pericentriolar matrix of the centrosome, essential for organizing the structure and function of centrosomes in vertebrate cells. It plays a central role in controlling centriole duplication, centrosome maturation, and microtubule nucleation. CEP57 is responsible for the engagement of centrioles during interphase and mitosis, acting as a structural hub for recruiting other critical PCM proteins such as Cep63 and Cep152, which are necessary for centrosome maturation and subsequent cell division fidelity[1][2][3][4][5]. CEP57 dynamically assembles via multivalent interactions and undergoes liquid–liquid phase separation, a process that is crucial for PCM expansion and the formation of microtubule asters. Truncating mutations in CEP57 cause mosaic-variegated aneuploidy syndrome, characterized by abnormal chromosome numbers and spindle defects[1][2]. Aberrant expression or depletion of CEP57 leads to centrosome amplification, PCM disorganization, and defects in mitotic spindle formation. Its function has implications for cancer biology due to its role in maintaining genomic integrity during cell division[3]. As of now, no drugs are known to specifically target CEP57, nor is it used as a clinical biomarker, but its role in cell division control makes it a relevant investigational target for mitosis-related disorders.

Other names

Centromatrix protein of 57 kDaKIAA0092TSP57TranslokinFGF2-interacting proteinTestis-specific protein 57MVA2PIG8proliferation-inducing protein 8