CEP164 (centrosomal protein 164) is a distal appendage protein of the mother centriole that plays a critical role in primary ciliogenesis and genomic stability. CEP164 functions as a scaffold protein at the centriole's distal appendages, where it interacts with TTBK2 through phase separation to recruit intraflagellar transport (IFT) machinery components essential for cilium initiation 12. CEP164 homodimerization via its central coiled-coil region is necessary for proper mother centriole localization and subsequent ciliogenesis 2. The protein operates within a hierarchical assembly pathway of distal appendage proteins, functioning alongside CEP83-SCLT1 as a critical module 3. CEP164 is widely expressed during embryonic and fetal development across multiple organ systems including kidneys, retina, and cerebellum, supporting its multi-system role 4. Mutations in CEP164 cause nephronophthisis-related ciliopathies and have been associated with Bardet-Biedl syndrome, primary congenital glaucoma, and atypical motile ciliopathy phenotypes 567. CEP164 interacts functionally with other ciliary proteins including INPP5E and CYP1B1, with co-inheritance of pathogenic variants associated with poorer clinical outcomes 78.