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Publication : Ultrastructural and physiological defects in the cochlea of the Mpv17 mouse strain. A comparison between young and old adult animals.

First Author  Meyer zum Gottesberge AM Year  2001
Journal  Hear Res Volume  156
Issue  1-2 Pages  69-80
PubMed ID  11377883 Mgi Jnum  J:106381
Mgi Id  MGI:3618426 Doi  10.1016/s0378-5955(01)00268-4
Citation  Meyer zum Gottesberge AM, et al. (2001) Ultrastructural and physiological defects in the cochlea of the Mpv17 mouse strain. A comparison between young and old adult animals. Hear Res 156(1-2):69-80
abstractText  Ultrastructural investigations were performed in young (approximately 2 months) and old (7 months) Mpv17-negative and wild-type mice. The onset, the severity and the pattern of the degeneration significantly differed between both mice strains. In the wild-type mouse strain the degenerative changes of the cochlear structures were similar to the aging pattern described for other species. In contrast, the Mpv17 mutants showed degenerative changes of the cochlear structures already at the age of 2 months. The degenerative changes were patchy arranged throughout the entire length of the cochlea and involved the organ of Corti as well as the stria vascularis epithelia with alterations of the basement membrane of the capillaries. The severe sensorineural hearing loss and degenerative changes of the cochlear structures indicate that cochlear structures, especially the outer hair cells and the intermediate cells of the stria vascularis, are vulnerable to the missing Mpv17 gene product.
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