PMID: 9447939Feb 3, 1998Paper

Loss of auditory function in transgenic Mpv17-deficient mice

Hearing Research
M MüllerR Klinke

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 compensa...Continue Reading

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Citations

Feb 11, 2012·Cell and Tissue Research·Angela-Maria Meyer zum GottesbergeStefan Hansen
Oct 22, 2009·Pigment Cell & Melanoma Research·Silvia Murillo-CuestaLluís Montoliu
Feb 27, 2009·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Harald WinterMarlies Knipper
Mar 21, 2013·Gene·Ling-Feng LuoWan-Xi Yang
Aug 1, 2006·The Journal of the Acoustical Society of America·Bradford J MayCynthia A Prosen
Sep 12, 2007·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·Ovidiu KönigMarlies Knipper
Dec 20, 2019·Journal of Comparative Physiology. A, Neuroethology, Sensory, Neural, and Behavioral Physiology·Johannes WetekamManfred Kössl
Sep 12, 2015·Biology Open·Reetta L HänninenHannes Lohi

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European Archives of Oto-rhino-laryngology : Official Journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : Affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery
Angela M Meyer zum GottesbergeHans Weiher
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