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Otogelin is a large secreted glycoprotein integral to the extracellular membranes of the inner ear, particularly the tectorial membrane[2][3]. It has a structure similar to, but distinct from, epithelial mucins, including several structural domains (e.g., von Willebrand factor domains, cysteine-rich C8 domains) and forms part of the fibrillar networks that ensure the structural integrity of acellular inner ear matrices[1][3]. Otogelin stabilizes and anchors the otoconial membranes and cupulae to the auditory and vestibular neuroepithelia, playing an essential role in hearing and balance. Mutations in the OTOG gene cause autosomal-recessive moderate-to-severe non-syndromic hearing loss and have been associated with variable clinical phenotypes, including occasional vestibular dysfunction and, in specific populations, familial forms of Meniere disease[2]. Otogelin is not a classic therapeutic target such as a receptor, enzyme, or ion channel, and there are no drugs known to act directly on otogelin[2][3].
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