OFD1 is an X-linked centriolar protein essential for primary ciliogenesis and centrosome organization. Functionally, OFD1 regulates centriole length and structure by stabilizing distal centriolar microtubules and promoting distal appendage formation 1. OFD1 recruits intraflagellar transport protein IFT88 to centrioles, enabling cilium assembly 1. The protein functions as a nucleation-promoting factor for Arp2/3 complex-mediated actin branching, linking cytoskeletal dynamics to cell cycle control 2. Mechanistically, OFD1 undergoes PKA-directed ubiquitylation via the TBC1D31/praja2 complex upon GPCR-cAMP stimulation, with proteolysis essential for proper ciliogenesis 3. OFD1 also regulates proteome balance through both proteasomal and autophagic pathways 4. Clinically, OFD1 mutations cause the ciliopathy spectrum ranging from orofaciodigital syndrome type I (X-linked dominant, male-lethal) to Joubert syndrome, primary ciliary dyskinesia, and retinitis pigmentosa 5. The phenotypic variability reflects distinct mutation-dependent effects on centriole elongation 1. Recent findings show OFD1 regulates BRCA1 expression; its inhibition induces BRCAness, creating PARP inhibitor sensitivity in pancreatic cancer 6. Additionally, OFD1 variants associate with autism spectrum disorder on the X chrX 7. Understanding OFD1's multifunctional role improves clinical management and reveals therapeutic opportunities beyond ciliopathies.