title: Loss of auditory function in transgenic Mpv17-deficient mice.
abstract: The transgenic mouse strain Mpv17 develops severe morphological degeneration of the inner ear and nephrotic syndrome at a young age (Meyer zum Gottesberge et al., 1996; Weiher et al., 1990). The audiograms (1-32 kHz) of Mpv17-negative mice were determined from auditory brain stem responses in young (2 months) and old (7 months) animals. Audiograms of age-matched wild-type mice with the same genetic background, but wild-type at the Mpv17 locus, were also determined. Furthermore, young Mpv17-negative mice that carried a human Mpv17 homologue gene were studied. NMRI mice served as a reference for normal hearing. Mpv17-negative mice suffer from severe sensorineural hearing loss as early as 2 months after birth. In the old Mpv17-negative mice no responses could be elicited at all. The 2 month old wild-type mice had normal audiograms, at 7 months only high threshold responses were seen. The poor audiograms of the Mpv17-negative mice are assumed to be the functional correlate of the morphological degeneration of the cochlea described earlier (Meyer zum Gottesberge et al., 1996). The finding that 2 out of 4 Mpv17-negative mice with the human Mpv17 gene had normal audiograms, shows that the gene inactivation can be functionally compensated by the human Mpv17 gene product.
authors.affiliation: Klinikum der J.W. Goethe-Universität, Physiologisches Institut III, Frankfurt am Main, Germany. m.mueller@em.uni-frankfurt.de
authors.forename: M
authors.initials: M
authors.lastname: Müller
authors.forename: J W
authors.initials: JW
authors.lastname: Smolders
authors.forename: A M
authors.initials: AM
authors.lastname: Meyer zum Gottesberge
authors.forename: A
authors.initials: A
authors.lastname: Reuter
authors.forename: R M
authors.initials: RM
authors.lastname: Zwacka
authors.forename: H
authors.initials: H
authors.lastname: Weiher
authors.forename: R
authors.initials: R
authors.lastname: Klinke
mesh_terms: D000367:Age Factors; D000818:Animals; D001299:Audiometry; D003051:Cochlea / Q000503:physiopathology; D016057:Evoked Potentials, Auditory, Brain Stem / Q000235:genetics* / Q000502:physiology; D018014:Gene Transfer Techniques; D006319:Hearing Loss, Sensorineural / Q000235:genetics* / Q000503:physiopathology; D006801:Humans; D008565:Membrane Proteins; D051379:Mice; D008822:Mice, Transgenic; D011506:Proteins / Q000235:genetics*
chemical_list: D008565:Membrane Proteins; C065292:Mpv17 protein, mouse; D011506:Proteins