behavior/neurological
• mice exhibit a body tilt
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• mutant mice are unable to remain at the surface of the water in a forced swim test whereas control mice are good swimmers
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• head- and body tilt can be observed at approximately 4 weeks of age with average onset 4.6 +/- 0.6 weeks
(J:82238)
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hearing/vestibular/ear
N |
• hearing is unaffected in mutant mice as assessed by ABR analysis
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• otoconia are absent in the utricle and saccule of newborn mutant mice
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• encephalographs indicate that mutant mice are nonresponsive even at maximum accelerations
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immune system
• mutant neutrophils do not produce superoxide in either the presence or absence of PMA, in contrast to controls
• Nox activity is completely absent from mutant neutrophils
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• at 3.5 days post-infection with 104 CFU of Burkholderia cepacia, spleen derived from mutant mice contain necrotic areas and increased numbers of polymorphonuclear cells within the marginal zone and the red pulp
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• at 3.5 days post-infection with 104 CFU of Burkholderia cepacia, mutant lung tissue is damaged by necrotizing pneumonia and alveolar spaces are filled with granulocytes, macrophages and cell debris
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• mutant animals innoculated intratrachially with 106 CFU of Burkholderia cepacia succumb to the infection within 3 days of infection with animals dying within 24 hours of the first signs of respiratory distress
• defective bacterial clearance in mutant mice is shown in lung homogenates; at 3.5 days post-infection with 104 CFU of Burkholderia cepacia, mutant lung homogenates contain more than 108 CFU of bacteria, while lung homogenates from control littermates do not contain any bacteria
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• at 3.5 days post-infection with 104 CFU of Burkholderia cepacia, mutant spleen contains large numbers of Burkholderia cepacia, whereas controls do not
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respiratory system
• at 3.5 days post-infection with 104 CFU of Burkholderia cepacia, mutant lung tissue is damaged by necrotizing pneumonia and alveolar spaces are filled with granulocytes, macrophages and cell debris
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hematopoietic system
• mutant neutrophils do not produce superoxide in either the presence or absence of PMA, in contrast to controls
• Nox activity is completely absent from mutant neutrophils
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