What We Do
We are interested in the gating of mechanically sensitive ion channels, which open in response to force on the channel proteins. We study these channels in vertebrate hair cells—the receptor cells of the inner ear, which are sensitive to sounds or accelerations. Hair cells have a bundle of actin-rich stereocilia rising from their apical surfaces. Mechanical deflection of a bundle changes the tension in fine "tip links" that stretch between the stereocilia; these filaments are thought to pull directly on the force-gated transduction channels to regulate their opening. One group of projects in the laboratory explores the structure, biophysics and function of the proteins that constitute the mechanosensation complex, with the goal of understanding the sensory transduction process in hair cells at an atomic level.
Many of these proteins are encoded by deafness genes. Our emerging understanding of these proteins, and the development of new methods for gene therapy by many labs, have led to the possibility of gene therapy for hereditary deafness. A second group of projects in this laboratory involves development of strategies for treating these disorders. We are focused especially on the most common form of hereditary deafness, and on two forms of Usher syndrome—a hereditary deafness and blindness—with the goal of bringing therapies to the clinic.