Centrosomal protein of 135 kDa (UniProt: Q66GS9; also known as Cep135, Centrosomal protein 4) is encoded by the CEP135 (also known as CEP4, KIAA0635) gene (Gene ID: 9662) in human. Cep135 is a centrosomal protein that is involved in centriole biogenesis. Human Cep135 is reported to contain two-stranded coiled-coil domains that are critical for microtubule binding. Cep135 acts as a scaffolding protein during early centriole biogenesis and is required for the targeting of centriole satellite proteins to centrosomes such as of PCM1, SSX2IP and CEP290 and recruitment of WRAP73 to centrioles. It also plays a role in centriole-centriole cohesion during interphase by acting as a platform protein for CEP250 at the centriole. Mutations in CEP135 gene are associated with autosomal recessive primary microcephaly-8, a disease characterized by intellectual disability, markedly reduced brain weight, and small cerebral cortex.
Synonyms: Centrosomal protoein of 135 kDa, Centrosomal protein 4
Application: Immunocytochemistry Analysis: A 1:100 dilution from a representative lot detected CEP135 in synchronized U2OS cells.Electron Microscopy Analysis: A representative lot detected CEP135 in Electron Microscopy applications (Kleylein-Sohn, J., et. al. (2007). Dev Cell. 13(2):190-202). Immunocytochemistry Analysis: A representative lot detected CEP135 in Immunocytochemistry applications (Kleylein-Sohn, J., et. al. (2007). Dev Cell. 13(2):190-202; Guderian, G., et. al. (2010). J Cell Sci. 123(Pt 13):2163-9; Sonnen, K.F., et. al. (2012). Biol Open. 1(10):965-76).
Other Notes: Concentration: Please refer to lot specific datasheet.