mortality/aging
• following infection with the types of organisms characteristic of human Chronic Granulomatous Disease (CGD) such as Staphylococcus and Lactobacillus, mice exhibit lethal infections not observed in wild-type mice
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immune system
• following intraperitoneal injection with thioglycollate, mice exhibit an abnormally exuberant inflammation recruiting twice as many leukocytes as in wild-type mice
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• impaired phagocytic response to Staphylococcal infection such that they produce no superoxide and kill Staphylococcus ineffectively
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• unlike wild-type mice, mice develop severe infections in response to types of organisms characteristic of human Chronic Granulomatous Disease (CGD) such as Staphylococcus and Lactobacillus
• infections are characterized by adenopathy, splenomegaly, and granulomatous pneumonia with neutrophils, plasma cells, lymphocytes, macrophages and multinucleated giant cells
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cardiovascular system
• hypoxic pulmonary vasoconstriction is significantly lower than for B6.129S-Cybbtm1Din or B6 controls
• esponse is primarily in the acute phase of the vasoconstrictor response while the sustained phase is similar to controls
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muscle
• hypoxic pulmonary vasoconstriction is significantly lower than for B6.129S-Cybbtm1Din or B6 controls
• esponse is primarily in the acute phase of the vasoconstrictor response while the sustained phase is similar to controls
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
chronic granulomatous disease | DOID:3265 |
OMIM:PS306400 |
J:28267 |