Recombinant Human Centrosomal protein of 135 kDa (CEP135), Truncated

Uniprot Id

Q66GS9

Target Species

Human

Target Name

CEP135

Target Full Name

Centrosomal protein of 135 kDa

Target Function

Centrosomal protein involved in centriole biogenesis. Acts as a scaffolding protein during early centriole biogenesis. Required for the targeting of centriole satellite proteins to centrosomes such as of PCM1, SSX2IP and CEP290 and recruitment of WRAP73 to centrioles. Also required for centriole-centriole cohesion during interphase by acting as a platform protein for CEP250 at the centriole. Required for the recruitment of CEP295 to the proximal end of new-born centrioles at the centriolar microtubule wall during early S phase in a PLK4-dependent manner.

Target Involvement

Microcephaly 8, primary, autosomal recessive (MCPH8)

Target Subcellular Location

Cytoplasm, cytoskeleton, microtubule organizing center, centrosome, centriole. Note=During centriole biogenesis, it is concentrated within the proximal lumen of both parental centrioles and procentrioles.

Target Protein Families

CEP135/TSGA10 family

Target Synonyms

centrosomal protein 135 kDa; centrosomal protein 135kDa; Centrosomal protein 4; Centrosomal protein of 135 kDa; centrosome protein 4; centrosome protein cep135; Cep135; CEP4; CP135_HUMAN

Target Background

This gene encodes a centrosomal protein, which acts as a scaffolding protein during early centriole biogenesis, and is also required for centriole-centriole cohesion during interphase. Mutations in this gene are associated with autosomal recessive primary microcephaly-8.