Analysis Tools
mortality/aging
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• numbers of homozygotes are subtly reduced compared to expected Mendelian rates from heterozygous intercrosses (wild-type:heterozygous:null, 508:881:409)
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behavior/neurological
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• abnormal startle response to acoustic stimuli; higher acoustic stimuli is required to increase startle response compared to controls with mutants showing a lower response to stimuli between 82 and 118 dB
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• mice show impaired coordination in the rotarod test in 1- and 3-day protocols
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• in open field, vertical activity is increased
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hearing/vestibular/ear
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• 9-month old mice have sensory epithelium abnormalities of the vestibular and auditory inner ear
• deposits of acellular material are present on the apical surface of the vestibular otoconial epithelia
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• outer hair cell degeneration occurs with collapse of the outer organ of Corti in the basal turn
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• some mice exhibit degeneration throughout the cochlea
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• semicircular canal organs show defects, albeit more subtle, similar to the acellular deposits on the vestibular otoconial epithelia
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• at 9 months, mice have reduced hearing sensitivity across frequencies
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nervous system
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• some mice exhibit degeneration throughout the cochlea
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• abnormal sensorimotor gating is exhibited
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hematopoietic system
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• all blood chemistry analyses are normal (hematocrit, hemoglobin content, platelet count, RBC and WBC counts, corpuscular parameters)
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• B cells do not express the glycan Neu5Ac on a lactosamine glycan chain on the cell surface, resulting in hyperresponsiveness to T-independent antigens
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• B cell proliferation is augmented compared to controls, in culture containing chicken and human serum, while proliferation is stronger in cultures containing fetal bovine serum
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• a significant increase in serum IgG1 level of Cmah-deficient cells
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• anti-CD3 stimulated T cells show reduced Interferon gamma and Il-4
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immune system
| N |
• development of immune cells appears to be grossly normal for B and T cell maturation, as well as B1 to B2 ratios, marginal zone to follicular B cell ratio, expression levels of IgM, MHC-II, and CD22, and activation marker profile
• germinal center formation in spleen in response to immunization with sheep red blood cells (SRBCs) is normal in mutants
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• B cells do not express the glycan Neu5Ac on a lactosamine glycan chain on the cell surface, resulting in hyperresponsiveness to T-independent antigens
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• B cell proliferation is augmented compared to controls, in culture containing chicken and human serum, while proliferation is stronger in cultures containing fetal bovine serum
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• a significant increase in serum IgG1 level of Cmah-deficient cells
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• anti-CD3 stimulated T cells show reduced Interferon gamma and Il-4
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• response to T-independent antigen like DNP-KLH is enhanced compared to controls, while T-dependent antigen response to DNP-Ficoll is not different
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• reduced levels of interferon gamma are produced by null T cells
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• reduced levels of Il-4 are produced by null T cells
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homeostasis/metabolism
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• wound repair is markedly delayed in mutants compared to wild-type mice, most noticeable in rate of wound closure between period of 4-9 days after wounding
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cellular
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• B cell proliferation is augmented compared to controls, in culture containing chicken and human serum, while proliferation is stronger in cultures containing fetal bovine serum
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