SCLT1 (sodium channel and clathrin linker 1) is a centriole distal appendage protein essential for primary cilium assembly and function. SCLT1 functions as a structural component of the distal appendage, operating within a CEP83-SCLT1 protein module critical for anchoring the mother centriole to the cell membrane and recruiting preciliary vesicles 1. It plays a primary role in ciliary assembly rather than disassembly, with depletion severely compromising all four key steps of ciliogenesis including intraflagellar transport initiation and CP110 removal 12. Loss of SCLT1 results in reduced ciliogenesis and impaired hedgehog signaling, particularly affecting limb bud development in a tissue-specific manner 3. Disorders of SCLT1 manifest across multiple ciliopathy phenotypes. Homozygous SCLT1 variants cause oral-facial-digital syndrome, Bardet-Biedl syndrome, and Senior-Løken syndrome, presenting with cystic kidney, cleft palate, polydactyly, and retinal degeneration 456. Sclt1-deficient mice develop cystic kidneys through activation of ERK and STAT3 signaling pathways, with STAT3 inhibition offering potential therapeutic benefit 4. Recent evidence extends SCLT1 involvement to nonsyndromic orofacial clefts through dominant pathogenic variants 7. The wide clinical spectrum reflects SCLT1's fundamental role in ciliary dynamics essential for developmental signaling and tissue homeostasis.