ANKS3 is a ciliary protein containing ankyrin repeats and a sterile alpha motif (SAM) domain that functions in kidney development and cilia-associated signaling. Primary function: ANKS3 is involved in vasopressin signaling in renal cilia and interacts with multiple nephronophthisis-associated proteins (NPHPs) including ANKS6, NPHP1, and the RNA-binding protein BICC1 1. Mechanism: ANKS3 interacts with ANKS6 through its SAM domain to form giant macromolecular complexes 2. ANKS3 also interacts with the kinase Nek7, which triggers modifications of ANKS3 and prevents Nek7 nuclear translocation, suggesting a role in regulating kinase localization 3. Additionally, ANKS3 displays RNA-binding properties and is recruited to stress granules upon cellular stress, indicating involvement in RNA processing responses 4. Disease relevance: ANKS3 mutations cause nephronophthisis, an autosomal recessive cystic kidney disease and leading cause of hereditary kidney failure in children 5. Knockdown of anks3 in zebrafish produces ciliary abnormalities, cyst formation, and laterality defects 5. Anks3 depletion disrupts amino acid and nucleotide metabolism, suggesting involvement in DNA damage responses 6. Clinical significance: ANKS3 dysfunction impairs normal renal development and represents a potential therapeutic target in cystic kidney diseases.