Seminar - Jeffrey Holt

 

Host: Martin Chalfie

 

Title: Structure, Function and Restoration of Mechanosensitive Ion Channels in Auditory Hair Cells

 

Abstract: Sensory transduction in the inner ear converts sound information into electrical signals that are transmitted to the brain. My lab has identified the elusive mechanosensitive ion channel critical for sensory transduction in auditory hair cells. For this seminar I will present structural and functional data supporting this conclusion. In addition to our basic science discoveries, our findings have translational relevance because the gene that encodes the hair cell transduction channel is the target of numerous genetic mutations that cause deafness in humans. To address genetic hearing loss, my lab has pioneered several forms of gene therapy. I will discuss our translational efforts to develop inner ear gene therapy, highlighting strategies for treating recessive and dominant forms of hearing loss. These include gene replacement, gene editing and base editing techniques. We have used these strategies to restore mechanosensory transduction in auditory and vestibular hair cells and hence restore hearing and balance function. We aim to develop these approaches into treatments for the benefit of patients with genetic inner ear disorders.