mortality/aging
• only 9% of pups born to heterozygote parents are homozyogtes instead of the expected 25%
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immune system
• clearance of senescent red blood cells in the spleen is impaired
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• by 16 weeks of age, spleen weight is about 50% more than in controls
• this increase in weight is attributable to an increase in stores of iron in the spleen
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• spleen red pulp macrophages (RPM), characterized by F4/80hiCD68+CD11blo expression with strong autofluorescence in flow cytometry, are selectively lost in these mice
• bone marrow chimera experiments demonstrate the defect in RPM generation is intrinsic to haematopoietic cells
• other macrophage population numbers, including marginal zone and metallophillic macrophages, are normal in these mice
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• iron levels are about double in the spleen compared to controls
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homeostasis/metabolism
• iron levels are about double in the spleen compared to controls
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hematopoietic system
• clearance of senescent red blood cells in the spleen is impaired
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• by 16 weeks of age, spleen weight is about 50% more than in controls
• this increase in weight is attributable to an increase in stores of iron in the spleen
|
• spleen red pulp macrophages (RPM), characterized by F4/80hiCD68+CD11blo expression with strong autofluorescence in flow cytometry, are selectively lost in these mice
• bone marrow chimera experiments demonstrate the defect in RPM generation is intrinsic to haematopoietic cells
• other macrophage population numbers, including marginal zone and metallophillic macrophages, are normal in these mice
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• iron levels are about double in the spleen compared to controls
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growth/size/body
• by 16 weeks of age, spleen weight is about 50% more than in controls
• this increase in weight is attributable to an increase in stores of iron in the spleen
|
cellular
• clearance of senescent red blood cells in the spleen is impaired
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