Analysis Tools
immune system
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• neutrophils do not efficiently reach peripheral inflammatory regions, likely contribution to infection susceptibility
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• mice often display splenomegaly
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• mutant neutrophils display increased superoxide anion production, even in absence of stimuli; stimuli such as fMLP, C5a, and zymosan increase superoxide anion production in both controls and null cells, but increase in null cells is substantially greater, while PMA stimulation results in equivalent superoxid production in null and heterozygous neutrophils
• despite increased anion production, phagocytosis of opsonized particles is equivalent to heterozygous
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• in transwell chemotaxis assays, migration of neutrophils from homozygotes in response to chemoattractants like C5a and Il8 is decreased; mutant neutrophils display only a 1.6-fold response in migratory activity compared to a 2.5- to 3.5-fold response in heterozygous neutrophils
• decrease is not due to changes in migration rates, but a reduced persistence of directionality
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• lungs of mice with splenomegaly are infiltrated by many lymphocytes, but very few neutrophils
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• after intranasal injection of Aspergillus fumigatus, hyperinflammation is detected in lungs of mutant mice, with presence of more fungal elements and infiltration by lymphocytes and macrophages, but not neutrophils, than in heterozygous mice
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hematopoietic system
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• neutrophils do not efficiently reach peripheral inflammatory regions, likely contribution to infection susceptibility
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• mice often display splenomegaly
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• histochemical analysis indicates presence of extramedullary hematopoiesis
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• mutant neutrophils display increased superoxide anion production, even in absence of stimuli; stimuli such as fMLP, C5a, and zymosan increase superoxide anion production in both controls and null cells, but increase in null cells is substantially greater, while PMA stimulation results in equivalent superoxid production in null and heterozygous neutrophils
• despite increased anion production, phagocytosis of opsonized particles is equivalent to heterozygous
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• in transwell chemotaxis assays, migration of neutrophils from homozygotes in response to chemoattractants like C5a and Il8 is decreased; mutant neutrophils display only a 1.6-fold response in migratory activity compared to a 2.5- to 3.5-fold response in heterozygous neutrophils
• decrease is not due to changes in migration rates, but a reduced persistence of directionality
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respiratory system
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• lungs of mice with splenomegaly are infiltrated by many lymphocytes, but very few neutrophils
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• in mice with splenomegaly, lungs are firm and stiff
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cellular
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• neutrophils do not efficiently reach peripheral inflammatory regions, likely contribution to infection susceptibility
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• in transwell chemotaxis assays, migration of neutrophils from homozygotes in response to chemoattractants like C5a and Il8 is decreased; mutant neutrophils display only a 1.6-fold response in migratory activity compared to a 2.5- to 3.5-fold response in heterozygous neutrophils
• decrease is not due to changes in migration rates, but a reduced persistence of directionality
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growth/size/body
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• mice often display splenomegaly
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