Analysis Tools
growth/size/body
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• older mice showed occasional hepatomegaly with extramedullary hematopoiesis
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• in older mice, 10 - 17 months of age
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• seen in mice from 7 to 17 months of age
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mortality/aging
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• 20% dead by 10 months of age
(J:72931)
• 50% dead by 11 months of age
(J:86202)
• 90% dead by 17 months of age
(J:86202)
|
nervous system
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• scattered apoptotic bodies are seen in the cerebral cortex, thalamus, and pyramidal neurons of the CA2/CA3 regions of the hippocampus in mice at 15 months of age
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• at 10 months of age, moderate amounts of autofluorescent deposits found in cells throughout brain, especially in deeper nuclei of cerebral cortex, hippocampus, and pons
(J:72931)
• autofluorescent bodies typical of neuronal ceroid lipofuscinosis are seen throughout the brain in mice at 15 months of age
(J:86202)
• autofluorescence appears as punctate yellow-green droplets with higher concentrations in the CA2/CA3 region of the hippocampus, the pons, and the lateral dorsal nucleus of the thalamus
(J:86202)
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• decreased by 10% at 15 months of age
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• atrophy of the white tracts in the core of the cerebellar folia
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• cerebral cortex atrophy at 15 months of age
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• compact with a moderate decrease in the dendritic arborization
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pigmentation
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• cells show bright autofluorescence
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• in the brain, pancreas, bone marrow, liver macrophages, testis, fundic and pyloric gastric glands, and retinal pigment epithelium
• at 15 months of age, the ultrastructure of the storage material in the brain appears as multilamellar membranous whorls, which in most cases appear within membranous vacuolar structures
• at 15 months of age, the ultrastructure of the storage material in the pancreas appears as large, irregular blocks, somewhat more dense than in the brain, with a multilamellar pattern surrounding occasional lipid-like droplets
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behavior/neurological
limb grasping
(
J:86202
)
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• in 50% of mice at 9 months of age and nearly 100% of mice at 13 months of age
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• at 10 months of age, apparent in 50% of mice and not as severe as Ppt1tm1Hof mice
(J:72931)
• by 15 months of age, manifest in all mice
(J:86202)
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endocrine/exocrine glands
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• fundic glands of the stomach contain abundant autofluorescent storage material
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• cells contain abundant autofluorescent storage material
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• enlarged, gelatinous and deeply pigmented, appearing orange to brown
• increase in the number of interstitial macrophages in the pancreas
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• massive autofluorescence is seen in the exocrine cells of the pancreas but autofluorescence is absent from the islet cells
• however, no signs of exocrine pancreatic dysfunction are detected
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• older mice (10 - 17 months) had zymogen granules replaced with large autofluorescent pigment granules
• no evidence of exocrine pancreas dysfunction, however
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• interstitial cells of the Leydig in the testes are distended and inclusions in the cytoplasm are visible as brown pigment that is brightly autofluorescent
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hematopoietic system
| N |
• despite disruption of normal compartments for hematopoiesis, normal peripheral blood counts
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• in older animals, evident in all spleens and some livers, with abundant neutrophilic and erythrocytic precursors and megakaryocytes
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• older mice had diffuse infiltration of macrophages, and large numbers of multinucleated giant cells
• large numbers of multinucleated giant cells that contain brightly autofluorescent material are seen in the marrow
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• increase in the number of interstitial macrophages in the pancreas
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• evident in older mice, loss of normal splenic architecture
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• in older mice, 10 - 17 months of age
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• seen in mice from 7 to 17 months of age
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• evident in older mice, loss of follicles containing B cells
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liver/biliary system
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• moderate autofluorescence is detected in the interstitial macrophages but not in the parenchymal cells
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• older mice showed occasional hepatomegaly with extramedullary hematopoiesis
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muscle
muscle spasm
(
J:86202
)
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• frequent myoclonic jerks without spontaneous seizures, beginning at 13 months of age
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digestive/alimentary system
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• massive autofluorescence is seen in the exocrine cells of the pancreas but autofluorescence is absent from the islet cells
• however, no signs of exocrine pancreatic dysfunction are detected
|
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• older mice (10 - 17 months) had zymogen granules replaced with large autofluorescent pigment granules
• no evidence of exocrine pancreas dysfunction, however
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• fundic glands of the stomach contain abundant autofluorescent storage material
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• cells contain abundant autofluorescent storage material
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immune system
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• increase in the number of interstitial macrophages in the pancreas
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• evident in older mice, loss of normal splenic architecture
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• in older mice, 10 - 17 months of age
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• seen in mice from 7 to 17 months of age
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• evident in older mice, loss of follicles containing B cells
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cellular
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• scattered apoptotic bodies are seen in the cerebral cortex, thalamus, and pyramidal neurons of the CA2/CA3 regions of the hippocampus in mice at 15 months of age
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reproductive system
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• interstitial cells of the Leydig in the testes are distended and inclusions in the cytoplasm are visible as brown pigment that is brightly autofluorescent
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vision/eye
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• cells show bright autofluorescence
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renal/urinary system
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• a fine dust-like autofluorescence is seen in transitional cells
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| neuronal ceroid lipofuscinosis | DOID:14503 |
OMIM:PS256730 |
J:72931 | |
