BMI Seminar // Jeff Holt: Function, Dysfunction and Restoration of Auditory Sensory Transduction

The sensory cells of the inner ear convert sound information into electrical signals that are transmitted to the brain.  A key molecule for this process is a mechanosensitive ion channel known as Transmembrane Channel One (TMC1).  TMC1 forms the pore of mechanosensory transduction channels in auditory hair cells.  Over a hundred genetic mutations have been identified in the TMC1 gene that cause hearing loss in humans.  To treat patients with TMC1 mutations we have developed gene replacement, gene editing and other novel strategies that restore auditory function in mice.  Our goal is to translate TMC1 therapies and other hearing loss therapies into clinical application.