Robert Fettiplace, PhD
Steenbock Professor of Neural and Behavioral Sciences, Department of Neuroscience, University of Wisconsin-Madison
Hearing Loss and hair cell death
Sensorineural deafness has two underlying origins, environmental and genetic. In both cases, a major cause is death of the cochlear hair cells. To understand the mechanisms of hair cell death, we have studied point mutations in the mechano-electrical (MET) channel protein, TMC1. These mutants surprisingly preserve MET channel function early in mouse neonatal development, but channel function is subsequently lost later in development, in the third to fourth postnatal week. This culminates in death of the hair cells and deafness. I shall describe two of the TMC1 mutants and possible experiments to document loss of MET channel function. I shall also discuss a possible link, involving reduced calcium influx through the MET channel leading to disorganization of hair bundle structure.