ANKS6 is a ciliary protein essential for renal and hepatic development. It localizes to the proximal compartment of the primary cilium 1, where it functions as a central hub protein linking multiple nephronophthisis (NPHP) pathway components including NEK8, INVS, and NPHP3 2. ANKS6 interacts with ANKS3 through sterile alpha motif (SAM) domains to form macromolecular complexes 34, and these assemblies are regulated by HIF1AN-mediated hydroxylation 2. In the kidney, ANKS6 loss causes cyst formation and nephronophthisis-16 (NPHP-16), characterized by progressive renal failure 52. In the liver, ANKS6 regulates bile duct morphogenesis by antagonizing Hippo signaling, promoting YAP1/TAZ transcriptional activity 1. Loss of ANKS6 leads to congenital hepatic fibrosis, ductal plate malformations, and periportal fibrosis through macrophage-driven inflammatory mechanisms 61. Biallelic ANKS6 mutations cause syndromic ciliopathies with multiorgan involvement including laterality defects, renal cysts, and hepatic fibrosis 2. Recent functional studies demonstrate that ANKS6 maintains proper polycystin-2 ciliary localization, as certain NEK8 variants reduce ANKS6-dependent signaling 7. These findings establish ANKS6 as a critical organizer of ciliary signaling networks controlling organ development.