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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
1-2
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pubmed:dateCreated |
1998-3-3
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pubmed:abstractText |
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.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:chemical | |
pubmed:status |
MEDLINE
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pubmed:month |
Dec
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pubmed:issn |
0378-5955
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:volume |
114
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
259-63
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pubmed:dateRevised |
2006-11-15
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pubmed:meshHeading |
pubmed-meshheading:9447939-Age Factors,
pubmed-meshheading:9447939-Animals,
pubmed-meshheading:9447939-Audiometry,
pubmed-meshheading:9447939-Cochlea,
pubmed-meshheading:9447939-Evoked Potentials, Auditory, Brain Stem,
pubmed-meshheading:9447939-Gene Transfer Techniques,
pubmed-meshheading:9447939-Hearing Loss, Sensorineural,
pubmed-meshheading:9447939-Humans,
pubmed-meshheading:9447939-Membrane Proteins,
pubmed-meshheading:9447939-Mice,
pubmed-meshheading:9447939-Mice, Transgenic,
pubmed-meshheading:9447939-Proteins
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pubmed:year |
1997
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pubmed:articleTitle |
Loss of auditory function in transgenic Mpv17-deficient mice.
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pubmed:affiliation |
Klinikum der J.W. Goethe-Universität, Physiologisches Institut III, Frankfurt am Main, Germany. m.mueller@em.uni-frankfurt.de
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pubmed:publicationType |
Journal Article,
Comparative Study,
Research Support, Non-U.S. Gov't
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