ADGRV1 (adhesion G protein-coupled receptor V1) is a large transmembrane receptor that functions as a G protein-coupled receptor involved in inner ear and retinal sensory function 1. The cleaved ADGRV1 beta-subunit couples with inhibitory G-alpha proteins (GNAI1/2/3) and constitutively inhibits adenylate cyclase activity through cAMP-PKA signaling, with the cleaved form showing stronger inhibitory effects than full-length ADGRV1 2. ADGRV1 is a core component of the ankle link complex (ALC), which assembles through liquid-liquid phase separation to organize developing stereocilia in inner ear hair cells 3. Within this complex, ADGRV1 regulates WHRN phosphorylation and subsequently controls USH2A stability through coordinated ubiquitination, essential for proper mechanoelectrical transduction 2. Disease-causing mutations in ADGRV1 disrupt ALC assembly and stability, impairing stereocilia organization and mechanotransduction. ADGRV1 mutations cause Usher syndrome 2C (USH2C), characterized by progressive hearing and vision loss 4, and account for less than 2% of recessive hearing loss cases in European populations 5. Additionally, ADGRV1 variants are associated with febrile seizures and epilepsy, with zebrafish models showing mutations affect neuromotor development and eye formation 6. Beyond sensory functions, ADGRV1 expression correlates with breast cancer prognosis and drug resistance mechanisms 7.