Otolith tethering in the zebrafish otic vesicle requires Otogelin and  -Tectorin

摘要: Otoliths are biomineralised structures important for balance and hearing in fish. Their counterparts the mammalian inner ear, otoconia, have a primarily vestibular function. otoconia form over sensory maculae attached to otolithic membrane, gelatinous extracellular matrix that provides physical coupling between otolith underlying epithelium. In this study, we identified two proteins required tethering zebrafish propose there at least stages process: seeding maintenance. The initial step, which precursor particles tether directly tips of hair cell kinocilia, fails occur einstein (eis) mutant. gene disrupted eis is otogelin (otog); mutations human OTOG recently been as causative deafness dysfunction (DFNB18B). At later larval stages, maintenance saccular macula dependent on tectorin alpha (tecta) function, rolling stones (rst) α-Tectorin (Tecta) major constituent tectorial membrane cochlea. Mutations TECTA can cause either dominant (DFNA8/12) or recessive (DFNB21) forms deafness. Our findings indicate composition otic membranes highly conserved mammals fish, reinforcing view an excellent model system study disease.