Science Daily: Structure of inner-ear protein is key to both hearing and inherited deafness

Thousands of microscopic hair cells located in the cochlea of the inner ear are extremely sensitive structures. Stereocilia are hair-like structures located on the hair cells and tip-links are filaments that connect the stereocilia in bundles. These structures are involved in the depolarization process, which ultimately leads to the excitation of the auditory nerve resulting in physiologic percept of sound.

ScienceDaily.com released an article on April 17, 2010 about new studies from the labs of David Corey, professor of neurobiology at Harvard Medical School and Howard Hughes Medical Institute investigator, and Rachelle Gaudet, associate professor of molecular and cellular biology at Harvard University's Faculty of Arts and Sciences. The group performed studies on one of two proteins, cadherin-23, that join to form each tip-link. Their studies show how mutations of the protein can make this otherwise extremely strong protein to weaken in its calcium bonding, resulting in inherited deafness. When weak, stress can cause this bond to "break in billionths of a second."

"Still image from a simulation that "stretches" the two end-most segments of the cadherin-23 protein, much as loud noise might do to its real-life counterpart in the inner ear. Such extreme stress can cause cadherin-23 to break in billionths of a second. (Credit: Marcos Sotomayor)"

Read the awesome article: Structure of inner-ear protein is key to both hearing and inherited deafness

via Science Daily