Identification of the protein product of the Coch gene (hereditary deafness gene) as the major component of bovine inner ear protein

In order to better understand the cause of hereditary hearing impairment, w e have performed a proteomic analysis of the inner ear proteins using two-d imensional gel electrophoresis. In the process of analysis, we have found v ery unique properties of the bovine homologue of the human COCH gene produc t. The COCH gene is responsible for one of the hereditary hearing impairmen ts, DFNA9, and was recently suggested to be a possible genetic factor contr ibuting to Meniere's disease. The Coch protein constitutes 70% of bovine in ner ear proteins and is composed of 16 different protein spots, with charge and size heterogeneity. Heterogeneity of this protein suggests that the Co ch gene is processed in several ways, at the transcriptional and/or posttra nslational level. Much knowledge has accumulated about the hereditary heari ng impairment genes; however, little research has been done regarding the p rotein products of those genes. This is the first report to characterize th e Coch protein. Study of the Coch protein might provide more information on the mechanism of hearing and vestibular disorders. (C) 2001 Elsevier Scien ce B.V. All rights reserved.