Analysis Tools
mortality/aging
|
• mutants die between 2 to 5 months of age, with about 75% dying at 4 months of age
(J:18424)
• originally mutants died by 4 months of age, however over time, the renal disease occurring in sucessive generations has become less aggressive and mutants can survive up to 1 year of age
(J:177825)
|
behavior/neurological
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• mutants develop hunched posture within 3-4 days of death
(J:18424)
• hunched posture appears around 8-9 months of age, shortly before death
(J:177825)
|
growth/size/body
weight loss
(
J:18424
)
|
• mutants start to lose weight within 3-4 days of death
|
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• progressive enlargement of the spleen
(J:18424)
|
immune system
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• perivenular granulocytic infiltration is seen in the liver
• granulocytic hyperplasia in the bone marrow and lymph nodes
|
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• mutants at terminal stages develop neutrophilia (only seen in the last 3-4 days of life)
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• scattered foci of plasmacytoid cells are present in the bone marrow and lymph nodes
• a thick perivascular cuff or plasmacytoid cells surround the renal blood vessel walls
|
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• mutants develop plasmacytosis of lymphoid organs (the spleen and lymph nodes), but not in other organs such as the liver, heart or lungs
(J:18424)
|
|
• progressive alteration of the spleen, with elevation of megakaryocyte numbers
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• progressive enlargement of the spleen
(J:18424)
|
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• IgG hypergammaglobulinemia
(J:18424)
|
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• increase in polyclonal IgG1 production
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• mutants exhibit increased concentrations of serum amyloid A
|
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• mutants at terminal stages exhibit enlargement of the lymph nodes
|
renal/urinary system
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• mutants with overt signs of disease exhibit proteinuria, mainly caused by IgG1
(J:18424)
|
|
• mutants at terminal stages exhibit whitening of the kidneys
• kidneys exhibit presence of protein casts in the tubuli and deposition of proteinaceous material
• mutants develop myeloma kidney
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• young animals exhibit an increase in the mesangial matrix of some kidney glomeruli
|
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• older mutants with hypergammaglobulinemia exhibit an increase in the number of damaged glomeruli and develop glomerulosclerosis
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• mutants that die exhibit tubular atrophy
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• tubules are dilated and filled with proteinaceous casts
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pale kidney
(
J:177825
)
|
• mutants exhibit loss of kidney function
(J:18424)
|
homeostasis/metabolism
amyloidosis
(
J:177825
)
|
• mutants exhibit amyloid protein deposits in the spleen, liver, and kidneys by 3 months of age
|
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• mutants exhibit increased concentrations of serum amyloid A
|
|
• mutants at terminal stages exhibit increased volume and clear signs of edema of the lungs
|
|
• mutants with overt signs of disease exhibit proteinuria, mainly caused by IgG1
(J:18424)
|
hematopoietic system
|
• increase in the number of megakaryocytes in the spleen and bone marrow
(J:18424)
• megakaryocytic hyperplasia in the bone marrow and lymph nodes
(J:177825)
|
|
• perivenular granulocytic infiltration is seen in the liver
• granulocytic hyperplasia in the bone marrow and lymph nodes
|
|
• mutants at terminal stages develop neutrophilia (only seen in the last 3-4 days of life)
|
|
• scattered foci of plasmacytoid cells are present in the bone marrow and lymph nodes
• a thick perivascular cuff or plasmacytoid cells surround the renal blood vessel walls
|
|
• mutants develop plasmacytosis of lymphoid organs (the spleen and lymph nodes), but not in other organs such as the liver, heart or lungs
(J:18424)
|
|
• progressive alteration of the spleen, with elevation of megakaryocyte numbers
|
|
• progressive enlargement of the spleen
(J:18424)
|
|
• IgG hypergammaglobulinemia
(J:18424)
|
|
• increase in polyclonal IgG1 production
|
respiratory system
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• mutants at terminal stages exhibit increased volume and clear signs of edema of the lungs
|
liver/biliary system
skeleton
|
• bones are fragile with osteopenia
|
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• thin cortices
|
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• loss of cancellous bone
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| amyloidosis | DOID:9120 | J:177825 | ||
