Analysis Tools
mortality/aging
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• begin to die from 7 months of age
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homeostasis/metabolism
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• young adults exhibit high transferrin saturation and a marked increase in serum ferritin levels
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• reduction in levels of lytic pancreatic enzymes such as amylase and elastase
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• iron overload in the liver, pancreas, kidney, heart, and brain, however zinc, copper, sodium, calcium, and phosphate levels are unaffected
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• iron accumulates in the heart, mainly in vascular smooth muscle cells
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• intestinal epithelium is iron depleted
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• progressive and marked iron accumulation in pancreatic tissue
• iron accumulates preferentially in the exocrine pancreas, and not in islets of Langerhans
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• decrease in splenic iron levels at 8 and 24 weeks of age
• selective iron depletion is seen within iron-recycling macrophages of the red pulp
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• accumulation of iron in the brain is seen in the choroid plexus
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• iron in kidney accumulates predominantly in the renal medulla
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• increase in hepatic iron levels, with preferential iron deposition in the pericentral areas of the liver lobules
• iron accumulates exclusively in hepatocytes
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hematopoietic system
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• decrease in splenic iron levels at 8 and 24 weeks of age
• selective iron depletion is seen within iron-recycling macrophages of the red pulp
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endocrine/exocrine glands
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• pancreas is dark brown
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• degeneration of the pancreatic acini starting at 21 weeks of age and worsening thereafter, with severe degeneration at 7 months
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• progressive and marked iron accumulation in pancreatic tissue
• iron accumulates preferentially in the exocrine pancreas, and not in islets of Langerhans
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growth/size/body
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• wasting is seen at 27 weeks of age
• death is preceded by a sudden and large loss of body weight, with mice humping the back and showing signs of sickness
• mice fed an iron-free diet show prevention of body weight loss and restored survival
• mice fed a chow supplemented with Pancrex, a compound used in pancreatic enzyme replacement therapy, partially reverses weight loss and improves survival
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digestive/alimentary system
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• degeneration of the pancreatic acini starting at 21 weeks of age and worsening thereafter, with severe degeneration at 7 months
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• intestinal epithelium is iron depleted
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cellular
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• iron accumulation in the pancreas triggers oxidative stress
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immune system
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• decrease in splenic iron levels at 8 and 24 weeks of age
• selective iron depletion is seen within iron-recycling macrophages of the red pulp
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liver/biliary system
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• increase in hepatic iron levels, with preferential iron deposition in the pericentral areas of the liver lobules
• iron accumulates exclusively in hepatocytes
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dark liver
(
J:215585
)
|
• liver is dark brown
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• increase in thiobarbituric acid reactive substances, indicating lipid peroxidation in the liver
• however, no histological evidence of liver damage
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cardiovascular system
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• iron accumulates in the heart, mainly in vascular smooth muscle cells
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nervous system
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• accumulation of iron in the brain is seen in the choroid plexus
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renal/urinary system
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• iron in kidney accumulates predominantly in the renal medulla
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| hemochromatosis type 4 | DOID:0111028 |
OMIM:606069 |
J:215585 | |
