CEP43 (centrosomal protein 43) is a centrosomal protein essential for ciliary function and microtubule anchoring. Its primary function involves anchoring microtubules to centrosomes, a critical role in maintaining centrosomal architecture 12. CEP43 is also required for ciliation, the process of forming functional cilia 32. Mechanistically, CEP43's N-terminal domain forms a homodimer that anchors to subdistal appendages through direct interaction with CEP350, while its C-terminal domain captures CEP19 above subdistal appendages, positioning it near the distal appendages 4. This positioning facilitates handoff of RabL2 from CEP19 to intraflagellar transport (IFT) complexes for ciliary entry, with CEP43-mediated interactions potentially priming the IFT machinery for ciliary translocation 4. Disease relevance: CEP43 variants have been identified as pathogenic in primary ciliopathies, a diverse group of rare disorders affecting multiple organ systems including the brain, eye, kidney, and skeleton 5. Clinical significance: Network-based genetic analysis identified CEP43 mutations in three previously unsolved ciliopathy cases, establishing it as a previously unrecognized ciliopathy gene and demonstrating its importance in systematic diagnosis of rare genetic diseases 5.