Based on limited published evidence, CPLANE2 is required for efficient primary cilia initiation by regulating late-stage cilia assembly steps, including mother centriole maturation and ciliary vesicle development 1. The protein facilitates axoneme extension through GTP-dependent interactions with CPLANE complex members and transition zone protein Fam92 1. CPLANE2 variants cause human ciliopathy presenting as Oral-Facial-Digital Syndrome, disrupting basal body docking and intraflagellar transport protein recruitment 1. The protein localizes to ciliary basal bodies and transition zones, regulating vesicle fusion and axoneme assembly through GTPase signaling.