G-protein coupled receptor which has an essential role in the development of hearing and vision (PubMed:16775142, PubMed:17567809, PubMed:20502675, PubMed:24191038). Couples to G-alpha(i)-proteins, GNAI1/2/3, G-alpha(q)-proteins, GNAQ, as well as G-alpha(s)-proteins, GNAS, inhibiting adenylate cyclase (AC) activity and cAMP production (PubMed:24191038, PubMed:24962568). Required for the hair bundle ankle formation, which connects growing stereocilia in developing cochlear hair cells of the inner ear (PubMed:16775142, PubMed:17567809). In response to extracellular calcium, activates kinases PKA and PKC to regulate myelination by inhibiting the ubiquitination of MAG, thus enhancing the stability of this protein in myelin-forming cells of the auditory pathway (PubMed:24191038). In retina photoreceptors, the USH2 complex is required for the maintenance of periciliary membrane complex that seems to play a role in regulating intracellular protein transport (PubMed:20502675). Involved in the regulation of bone metabolism (PubMed:22419726). Cleaved ADGRV1 beta-subunit couples with G-alpha(i)-proteins, GNAI1/2/3, and constitutively inhibits adenylate cyclase (AC) activity with a stronger effect than full ADGRV1. Forms a heterodimer, consisting of a large extracellular region (alpha subunit) non-covalently linked to a seven-transmembrane moiety (beta subunit) (PubMed:24962568). Interacts (via the cytoplasmic region) with PDZD7 (PubMed:24962568, PubMed:23055499). Component of USH2 complex, composed of ADGRV1, PDZD7, USH2A and WHRN (PubMed:20502675, PubMed:25406310). Interacts with USH2A and WHRN (PubMed:20502675, PubMed:23055499). Interacts (via the cytoplasmic region) with MYO7A (via MyTH4-FERM domains) (PubMed:17567809). Localizes at the ankle region of the stereocilia (PubMed:16775142, PubMed:27525485). In photoreceptors, localizes at a plasma membrane microdomain in the apical inner segment that surrounds the connecting cilia called periciliary membrane complex (PubMed:20502675, PubMed:24334608). Expressed by oligodendrocytes. In midbrain, enriched in the myelinated regions of the superior and inferior colliculi (PubMed:24191038). In the cochlea, expressed in developing hair cells (PubMed:16775142, PubMed:20502675, PubMed:17567809). Expressed by photoreceptors in the retina (PubMed:20502675). High level expression restricted to the developing central nervous system and eye. At mid-gestation expression is prominent in the ventricular zone and in the eye. At late gestation expression declines (PubMed:11606593). In the inner ear, highly expressed at 17 dpc at the base of emerging hair bundle and on peripheral subpopulation of microvilli located at the neural edge of the hair cell apical surface. At P0-P1, the expression extends from the very base of the stereocilia. From P4 to P9, expression becomes restricted, forming a basal band-like pattern at the ankle link level above the stereocilia. From P12 onward, no longer detectable in the cochlear hair cells (PubMed:16775142, PubMed:20502675). In the utricle of the vestibular system, the expression persists for longer, being present at both P4 and P11, but no longer detectable by P18 (PubMed:16775142). The 7 transmembrane domain is required in hair cells for the hair bundle ankle formation. Autoproteolytically cleaved into 2 subunits, an extracellular alpha subunit and a seven-transmembrane subunit. Mice display hearing loss and audiogenic epilepsy (PubMed:15606908, PubMed:17329413). Audiogenic epilepsy syndrome is an autosomal recessive mutation, characterized by generalized self-sustained convulsive seizures in which acoustics stimulations evoke wild running, tonic flexion and extension (PubMed:15606908). They are induced by exposing animals to a loud noise during the early stages of their development (PubMed:15606908). Mutants show a decreased bone mineral density (PubMed:22419726). Secreted. Secreted. Belongs to the G-protein coupled receptor 2 family. Adhesion G-protein coupled receptor (ADGR) subfamily.