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a Location of pathogenic mutations in Claudin-14. Colored amino acid residues indicate previously reported claudin-14 mutations. Arrow indicates position of CLDN14 c.488C>T [p.(Ala163Val)]. Adapted from: Bashir et al. (2013), b Cross-sectional diagram illustrating the anatomical location of the cochlear canals and their respective ionic composition. *CLDN14 expression, c Schematic diagram demonstrating the molecular structure of tight junctions
