Analysis Tools
mortality/aging
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• no mice survive for more than 63 days
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growth/size/body
weight loss
(
J:285931
)
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• mice weight less already at weaning, barely gain weight after weaning and start to lose weight from week 5 onwards
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• spleen in enlarged in 35 day old mice
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hematopoietic system
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• thymus cellularity is reduced in 35 day old mice
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• spleen in enlarged in 35 day old mice
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• however, the total amount of hemoglobin is not changed and hematocrit and platelet counts are normal
• average mass of hemoglobin in erythrocytes is reduced
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• granulocyte infiltration in the spleen, lymph node and thymus
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• focal infiltration of white matter tracts by ballooned macrophages are seen in the corpus callosum, the subcortical white matter of the hemispheres, the corticospinal tract, the optic tract, the cerebellar white matter, the pyramidal tract and all spinal tracts
• perivascular macrophage cuffs are present in the cerebellum and in the meninges
• macrophage accumulation is also seen in the olfactory bulb
• meninges show a scattered, diffuse infiltration by monocytes/macrophages
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• foamy macrophage infiltration in the spleen, lymph node, thymus, liver, and joints
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• lymphoid organs are largely infiltrated with monocytic cells
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• the number of circulating lymphocytes is reduced
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• B-lymphocytes are decreased in peripheral blood
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• T-lymphocytes are decreased in peripheral blood
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• CD4/CD8 double positive T cells are reduced in peripheral blood resulting in a decrease of CD4 and CD8 single positive T cells
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• expansion of the early hematopoietic stem and progenitor cell compartment and the lineage committed progenitor cell compartment
• however, the long-term stem cells are unaffected
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• absolute number of splenocytes is reduced in 35 day old mice
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homeostasis/metabolism
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• blood urea nitrogen is elevated in 6 week old mice, indicating a decrease of glomerular filtration rate
• however, no histological abnormalities are seen in the kidney
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• serum levels of the cytokines MCP-1, MIP-1 alpha, VEGF and IP-10 are elevated
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• serum levels of IP-10 are elevated
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• glutamate-pyruvate transaminase levels increase over time and are significantly elevated in 6 week old mice
• however, total serum protein, bilirubin levels, and albumin levels are not altered
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• glutamic-oxaloacetic transaminase levels increase over time and are significantly elevated in 6 week old mice
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• creatinine phosphokinase levels are increased over time, indicating damage to muscle cells
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• lactate dehydrogenase levels are increased over time, indicating damage to muscle cells
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• lungs show an increase of C16- and C24:1-sphingomyelins and decrease of C18-, C20-, and C24-sphingomyelins
• liver shows elevation of all sphingomyelin species, except C22 and C24 species
• spleen shows an increase of C16-, C22-, C24- and total sphingomyelin, but reduced C20-species
• kidneys show an elevation of all sphingomyelin species, except C24- and C24-1 species which are reduced
• in muscle and brain tissue, all sphingomyelin levels and total sphingomyelin are increased
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• total ceramide levels are elevated in all tested tissues
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• sphingosine levels are elevated 4-fold in the liver
• however, spingosine-1-phosphate levels are not altered
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immune system
|
• thymus cellularity is reduced in 35 day old mice
|
|
• spleen in enlarged in 35 day old mice
|
|
• granulocyte infiltration in the spleen, lymph node and thymus
|
|
• focal infiltration of white matter tracts by ballooned macrophages are seen in the corpus callosum, the subcortical white matter of the hemispheres, the corticospinal tract, the optic tract, the cerebellar white matter, the pyramidal tract and all spinal tracts
• perivascular macrophage cuffs are present in the cerebellum and in the meninges
• macrophage accumulation is also seen in the olfactory bulb
• meninges show a scattered, diffuse infiltration by monocytes/macrophages
|
|
• foamy macrophage infiltration in the spleen, lymph node, thymus, liver, and joints
|
|
• lymphoid organs are largely infiltrated with monocytic cells
|
|
• the number of circulating lymphocytes is reduced
|
|
• B-lymphocytes are decreased in peripheral blood
|
|
• T-lymphocytes are decreased in peripheral blood
|
|
• CD4/CD8 double positive T cells are reduced in peripheral blood resulting in a decrease of CD4 and CD8 single positive T cells
|
|
• absolute number of splenocytes is reduced in 35 day old mice
|
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• serum levels of the cytokines MCP-1, MIP-1 alpha, VEGF and IP-10 are elevated
|
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• serum levels of IP-10 are elevated
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• lymph node cellularity is reduced in 35 day old mice
• however, bone marrow cellularity is not altered
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• mice exhibit lung inflammation, with neutrophil and myeloid-derived suppressor cell infiltration and decreased dendritic cell and B-cell numbers but unaltered macrophage or T cell numbers
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limbs/digits/tail
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• synovial hyperplasia in knee joints due to the presence of foamy macrophages
• however, cartilage loss, bone erosion or exudation into the joint cavity are not seen
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liver/biliary system
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• mice develop pericentral and pericellular hepatic fibrosis
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muscle
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• muscles show some atrophic fibers in 6 week old mice
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• muscles show centrally displaced nuclei in 6 week old mice
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• mice show some indicators of muscular dystrophy, showing progressive wasting, scoliosis, and waddling gait
• creatinine phosphokinase and lactate dehydrogenase levels are increased over time, indicating damage to muscle cells
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nervous system
astrocytosis
(
J:285931
)
respiratory system
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• mice exhibit lung inflammation, with neutrophil and myeloid-derived suppressor cell infiltration and decreased dendritic cell and B-cell numbers but unaltered macrophage or T cell numbers
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skeleton
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• synovial hyperplasia in knee joints due to the presence of foamy macrophages
• however, cartilage loss, bone erosion or exudation into the joint cavity are not seen
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cellular
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• foamy macrophage infiltration in the spleen, lymph node, thymus, liver, and joints
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endocrine/exocrine glands
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• thymus cellularity is reduced in 35 day old mice
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behavior/neurological
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• mice show waddling gait
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Farber lipogranulomatosis | DOID:0050464 |
OMIM:228000 |
J:285931 | |
