Centrosomes are the major microtubule-organizing centers of mammalian cells. They are composed of a centriole pair and surrounding microtubule-nucleating material termed pericentriolar material (PCM). Bipolar mitotic spindle assembly relies on two intertwined processes: centriole duplication and centrosome maturation. Failure to properly orchestrate centrosome duplication and maturation is subsequently linked to spindle defects, which can result in aneuploidy and promote cancer progression. The CEP76 (centrosomal protein of 76 kDa) gene encodes a protein of 659 amino acids. The CEP76 protein is homologous among several species including human, mouse, rat and xenopus, suggesting a conserved function.