mortality/aging
• 20% of mice die by 5 months of age
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• fewer than expected mice survive until P10
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hematopoietic system
• under deoxygenated conditions, more than 95% of cells sickle in less than 50 seconds and many exhibit profusion of spicules unlike wild-type cells
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• delay times for polymerization of hemoglobin are shorter than in Hbbd3th/Hbbd3th Tg(LCR-HBA2,LCR-HBB)11Cos mice
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• compared with Hbbd3th/Hbbd3th Tg(LCR-HBA2,LCR-HBB)11Cos mice
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• mice exhibit spleen fibrosis unlike wild-type mice
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• mice exhibit spleen congestion unlike wild-type mice
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homeostasis/metabolism
• after 7 hours of water deprivation, mice exhibit a reduced capacity to concentrate urine compared with similarly treated wild-type mice
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cardiovascular system
• mice exhibit dilation of renal afferent vessels unlike wild-type mice
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• two younger mice exhibit congestion in the brain unlike wild-type mice
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• mice exhibit kidney glomerular congestion unlike wild-type mice
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• mice exhibit lung congestion unlike wild-type mice
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• mice exhibit spleen congestion unlike wild-type mice
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• mice exhibit intra-alveolar hemorrhage unlike wild-type mice
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liver/biliary system
• mice exhibit multifocal ischemic infarcts in the liver with dilation of the central vein and a preserved rim of viable cells around the central vein unlike wild-type mice
• older mice exhibit liver scarring and fibrosis with collapsed areas unlike wild-type mice
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• in older mice
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nervous system
• two younger mice exhibit congestion in the brain, occasional red neurons, and rare pyknotic neurons unlike wild-type mice
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• two younger mice exhibit congestion in the brain unlike wild-type mice
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• mice exhibit red neurons unlike in wild-type mice
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renal/urinary system
• mice exhibit dilation of renal afferent vessels unlike wild-type mice
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• mice exhibit kidney glomerular congestion unlike wild-type mice
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• after 7 hours of water deprivation, mice exhibit a reduced capacity to concentrate urine compared with similarly treated wild-type mice
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• mice exhibit focal fibrosis in the medulla and the cortical-medullary junction unlike wild-type mice
• one mouse exhibits cortical fibrosis
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respiratory system
• mice exhibit lung congestion unlike wild-type mice
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• mice exhibit intra-alveolar hemorrhage unlike wild-type mice
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• in two older mice with some platelet thrombi
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• in two older mice
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immune system
• mice exhibit spleen fibrosis unlike wild-type mice
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• mice exhibit spleen congestion unlike wild-type mice
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growth/size/body
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
sickle cell anemia | DOID:10923 | J:94190 |