SDCCAG8 is a centrosomal protein essential for ciliogenesis and Hedgehog signaling. Mechanistically, SDCCAG8 mediates RABEP2 centrosomal localization, which is critical for primary cilia formation 1. The protein also regulates pericentriolar material recruitment and interacts with PCM1 to control centrosomal function and neuronal migration during cortical development 2. SDCCAG8 is associated with multiple ciliopathies: mutations cause Bardet-Biedl syndrome 16 and Senior-Loken syndrome 7, characterized by progressive kidney and retinal degeneration 34. Disease penetrance in SDCCAG8-related kidney failure is 100%, with median onset at 12.5 years 3. Beyond renal-retinal disease, SDCCAG8 deficiency impairs primary ciliogenesis and cilium-dependent signaling, causing altered transcription of genes enriched in neurodevelopmental processes 5. Common variants associate with schizophrenia and cognitive function through altered gene regulation rather than severe structural defects 5. Recent proteomic studies identified SDCCAG8 as a potential therapeutic target for chr1 kidney disease 6. Additionally, SDCCAG8 regulates DNA damage response signaling in renal tissue 7, suggesting multiple mechanisms contributing to ciliopathy pathogenesis.