CEP295 is a centriole-enriched microtubule-binding protein essential for centriole biogenesis and centrosome function 1. During the S/G2 phase of the cell cycle, CEP295 is recruited to procentrioles and mediates distal centriole elongation in a CPAP- and CEP120-dependent manner, acting downstream of the initial cartwheel assembly 1. CEP295 directly interacts with centriolar microtubules and recruits structural proteins including POC1B, POC5, and CEP135 to the distal centriole, while also regulating post-translational modifications such as acetylation and glutamylation of centriolar microtubules 1. The protein functions as a molecular scaffold during centriole-to-centrosome conversion, a maturation process required for centrosome duplication and pericentriolar material organization 2. Loss-of-function mutations in CEP295 cause Seckel-like syndrome characterized by primary microcephaly, developmental delay, intellectual disability, short stature, and craniofacial abnormalities 3. Mechanistically, CEP295 depletion reduces centriole and centrosome numbers, triggers p53-dependent cell cycle arrest, and causes primary ciliary defects 3. CEP295 has also been identified as a candidate gene in broader ciliopathy cohorts 4, suggesting roles in ciliogenesis beyond centrosome function.