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Publication : A substrain of NZB mouse as an animal model of autoimmune inner ear disease.

First Author  Sone M Year  1995
Journal  Hear Res Volume  83
Issue  1-2 Pages  26-36
PubMed ID  7607988 Mgi Jnum  J:25486
Mgi Id  MGI:73034 Doi  10.1016/0378-5955(94)00189-w
Citation  Sone M, et al. (1995) A substrain of NZB mouse as an animal model of autoimmune inner ear disease. Hear Res 83(1-2):26-36
abstractText  A substrain of an autoimmune-prone mouse, NZB/kl, was found to show spontaneous elevation of the auditory brainstem response (ABR) threshold with age. Morphological examination of the inner ear in NZB/kl mice with high ABR thresholds revealed pathological changes confined to the stria vascularis, including marked thickening of the capillary basement membrane which contained many foamy structures, and vacuolar degeneration of the intermediate cells. Circular or granular IgM deposits and some IgG deposits were found in the stria vascularis in the mice with high ABR thresholds, suggesting that deposits of immune complexes (mainly IgM antibodies) could cause strial damage that resulted in the ABR threshold elevation. Another substrain of NZB mice, NZB/san, showed lower levels of IgM immune complexes and anti-ss DNA antibodies, and did not develop either inner ear morphological changes or a high ABR threshold. NZB/kl mice may provide a useful animal model for studying the mechanism of autoimmune inner ear disease.
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