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Publication : Cochlear and retinal degeneration in the tubby mouse.

First Author  Ohlemiller KK Year  1995
Journal  Neuroreport Volume  6
Issue  6 Pages  845-9
PubMed ID  7612867 Mgi Jnum  J:26067
Mgi Id  MGI:73689 Doi  10.1097/00001756-199504190-00005
Citation  Ohlemiller KK, et al. (1995) Cochlear and retinal degeneration in the tubby mouse. Neuroreport 6(6):845-9
abstractText  A number of autosomal recessive syndromes feature both sensorineural hearing loss and retinal degeneration. The mouse mutant tubby also combines hearing loss with progressive retinal degeneration, and thus may constitute a useful model of one form of human sensorineural deafness/retinal dystrophic syndrome. It has not been directly demonstrated that the hearing loss in this mouse involves the cochlea, however. We have examined the cochleas of adult tubby mice using light microscopy. The tubby cochlea shows pronounced degeneration of the organ of Corti and loss of afferent neurons in the base, with relative sparing of the apex. Our findings support the tubby mouse as a model of human sensorineural deafness/retinal dystrophic syndrome. Possible human counterparts include Usher's, Alstrom's, and Bardet-Biedl syndromes.
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