Centrosomal protein 135 (CEP135)

Centrosomal protein 135 (CEP135) is a coiled-coil protein found in the centrosome throughout the cell cycle and is highly conserved across eukaryotes[2][4]. It localizes specifically to the cartwheel within centrioles and functions as a structural scaffold during the very earliest stages of centriole assembly[1][3][4][5]. CEP135 stabilizes the connection between inner cartwheel components (e.g., SAS-6) and other centriolar proteins, maintaining the cohesion and integrity of centrioles[1][3][4][5]. While essential for proper centriole structure in many organisms, CEP135 is not strictly required for centriole duplication in all species. In vertebrates and mammals, its deficiency results in mild abnormalities in centriole numbers, increased monopolar spindles, and abnormal centriole ultrastructure[1][3][4]. Mutations in CEP135 cause autosomal recessive primary microcephaly (MCPH8), linking it to neurodevelopmental disorders[5]. There is no evidence that CEP135 is itself a druggable target, nor are there known drugs that interact directly with it; its principal significance is as a structural determinant essential for normal cell division, and as a gene whose mutation leads to specific human diseases[5].

Other names

CEP135Centrosomal protein of 135 kDaKIAA0635CEP4FLJ13621MCPH8 (associated with disease phenotype)Centrosomal protein 4Centrosome protein cep135