Matthew Hinsley '07

Myosin VII A and Deafness

The understanding of the genetics in hearing loss is an area of avid interest but of somewhat limited knowledge. Specifically, mutation of the gene encoding myosin VII A contributes to a substantial proportion of syndromic and non-syndromic congenital deafness. However, comprehension of the fundamental role of myosin VII in inner ear hair cell function remains enigmatic. Further insight may be gained by artificially creating a disease phenotype which could be used for scrutiny in eliciting a function. Removing the myosin VIIA proteins at the cellular level would create such a phenotype.

The goal of this study, therefore, is to demonstrate the ability to knock-out myosin VII A in neonatal rat outer hair cells using highly specialized small-interfering RNA. This would provide a useful model for simulating genetic hearing loss and help unfold the role of myosin VII A in sound transmission. Our method involved dissecting out neonatal rat cochlea and culturing the outer hair cells on post-natal days 3-9. Small interfering RNA (siRNA) was then introduced in-vitro into the cells. The siRNA was injected into the outer hair cells using the microprojectile approach ("Gene Gun" mediated gene transfer). Beta-actin was employed as a control. The genetically altered cells had a reduction or absence of myosin VII A, as demonstrated using immunolabeling methods along with light microscopy.

The significance of this study was in future applications of the model to the discovery of the function of myosin VIIA, and the potential use of this model in discovering diagnostic or treatment of myosin VIIA associated deafness.

Administration - Maps - Affiliated Hospitals - Med Student Information - UW Home

Otolaryngology Surgery University of Wisconsin Department of Surgery
First published: 07/15/02 Last updated: 11/24/09 webmaster@surgery.wisc.edu
Copyright © 2005 The Board of Regents of the University of Wisconsin System