Usher Syndrome Protein 1G (USH1G)

USH1G encodes a protein that contains three ankyrin domains, a class I PDZ-binding motif and a sterile alpha motif. The encoded protein interacts with harmonin, which is associated with Usher syndrome type 1C. This protein plays a role in the development and maintenance of the auditory and visual systems and functions in the cohesion of hair bundles formed by inner ear sensory cells. Mutations in this gene are associated with Usher syndrome type 1G (USH1G).deduced 460-amino acid protein contains 3 ankyrin-like domains near the N terminus, a central region, and a SAM domain and a PDZ-binding motif at the C terminus. The human protein shares 96% sequence identity with the mouse protein.