title: Tricellular Tight Junctions in the Inner Ear.
abstract: Tight junctions (TJs) are structures that seal the space between the epithelial cell sheets. In the inner ear, the barrier function of TJs is indispensable for the separation of the endolymphatic and perilymphatic spaces, which is essential for the generation and maintenance of the endocochlear potential (EP). TJs are formed by the intercellular binding of membrane proteins, known as claudins, and mutations in these proteins cause deafness in humans and mice. Within the epithelial cell sheet, however, a bound structure is present at the site where the corners of three cells meet (tricellular tight junctions (tTJs)), and the maintenance of the barrier function at this location cannot be explained by the claudins alone. Tricellulin and the angulin family of proteins (angulin-1/LSR, angulin-2/ILDR1, and angulin-3/ILDR2) have been identified as tTJ-associated proteins. Tricellulin and ILDR1 are localized at the tTJ and alterations in these proteins have been reported to be involved in deafness. In this review, we will present the current state of knowledge for tTJs.
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Graduate School of Medicine, Kyoto University, Kyoto 606-8507, Japan.
authors.forename: Shin-Ichiro
authors.identifier: 0000-0001-5643-0470
authors.initials: S
authors.lastname: Kitajiri
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Graduate School of Medicine, Kyoto University, Kyoto 606-8507, Japan.
authors.forename: Tatsuya
authors.identifier: 0000-0002-0395-4382
authors.initials: T
authors.lastname: Katsuno
mesh_terms: D003638:Deafness / Q000378:metabolism*; D007758:Ear, Inner; D004847:Epithelial Cells / Q000378:metabolism*; D006801:Humans; D062794:MARVEL Domain Containing 2 Protein / Q000378:metabolism*; D008297:Male; D062725:Tight Junction Proteins / Q000378:metabolism*; D019108:Tight Junctions / Q000378:metabolism*
chemical_list: D062794:MARVEL Domain Containing 2 Protein; D062725:Tight Junction Proteins