homeostasis/metabolism
• basal cholesterol levels are increase compared to wild-type controls probably as a result of differences in strain background
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Allele Symbol Allele Name Allele ID |
Psen1tm1Lpr targeted mutation 1, Laurent Pradier MGI:3510457 |
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Summary |
3 genotypes
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• basal cholesterol levels are increase compared to wild-type controls probably as a result of differences in strain background
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• accelerated rate of A-beta deposition starting at 2.5 months of age
• by 6 months of age deposition has become widespread and numerous in cortical, hippocampal and thalamic areas
• astrocytic and microglial activation around amyloid plaques
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• marked reduction in thickness of the hippocampal pyramidal cell layer by 10 months of age
• no amyloid plaques in the CA1/2 pyramidal cell layer
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• accelerated rate of A-beta deposition starting at 2.5 months of age
• by 6 months of age deposition has become widespread and numerous in cortical, hippocampal and thalamic areas
• astrocytic and microglial activation around amyloid plaques
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
Alzheimer's disease | DOID:10652 | J:93770 | ||
Alzheimer's disease 3 | DOID:0110042 |
OMIM:607822 |
J:93770 |
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• some plaques are detected at 2 months of age in the cervical spinal cord and plaques increase with age
(J:128106)
• plaque formation is higher in the cervical spinal cord compared to the thoracic and lumbar regions
(J:128106)
• plaques are almost exclusively found in the gray matter
(J:128106)
• amyloid plaques are located frequently within the granular cell layer of the dentate gyrus
(J:139736)
• significant plaque formation is detected in the frontal cortex by 2 months of age
(J:146342)
• from 6 to 12 months of age plaques become more densely packed
(J:146342)
|
• amyloid beta plaques form but no intracellular aggregates are seen
• numerous plaques form between 2 and 6 months of age and the pathology increases with age
• unlike in the frontal cortex, no neuronal loss is detected in the thalamus
|
• the granule cell layer is less densely packed at 12 months of age compared to 2 months of age
• the granule cell border is often frayed
• ectopic cells that are often orientated towards extracellular amyloid deposits can be detected at 2 months of age
• amyloid plaques are located frequently within the granular cell layer of the dentate gyrus
• decrease in the number of dentate gyrus granule cells at 12 months of age
|
• decrease in the number of dentate gyrus granule cells at 12 months of age
|
• massive intra- and extra-cellular amyloid beta deposits are detected in the frontal cortex at as early as 1.5 months of age, before significant plaque formation is detected
• by 6 months of age intracellular accumulations are no longer a prominent pathological feature
• a 28% and 35% loss of neurons is seen in the frontal cortex at 6 and 12 months of age, respectively, compared to age-matched controls
• frontal cortex volume is reduced compared to controls at 6 and 12 months of age
|
• thickened and irregularly shaped neurites are seen in the medulla at 14 months of age
|
• axonal spheroids are detected in pons and cortical areas at 6 months of age and in the spinal cord mostly along the anterior margin of the ventral horn and less frequently in the intermediolateral and dorsal horn
• at 10 and 14 months of age axonal spheroids are detected in pons, cortex, medulla, midbrain, hippocampus, corpus callosum and striatum
• larger axonal varicosities are seen in the medulla at 14 months of age
• large axonal dilatations are localized in white matter fiber tracts in close vicinity to the ventral horn in the spinal cord
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• dilated axons often display thinned or focally absent myelin sheaths
• increased deposition of myelin ovoids in the white matter
|
• a 28% and 35% loss of neurons is seen in the frontal cortex at 6 and 12 months of age, respectively, compared to age-matched controls
|
• some plaques are detected at 2 months of age in the cervical spinal cord and plaques increase with age
(J:128106)
• plaque formation is higher in the cervical spinal cord compared to the thoracic and lumbar regions
(J:128106)
• plaques are almost exclusively found in the gray matter
(J:128106)
• amyloid plaques are located frequently within the granular cell layer of the dentate gyrus
(J:139736)
• significant plaque formation is detected in the frontal cortex by 2 months of age
(J:146342)
• from 6 to 12 months of age plaques become more densely packed
(J:146342)
|
• significant decrease in plasma cholesterol between 2 and 6 months of age that is not seen in wild-type controls or either single transgenic mice
|
• basal cholesterol levels are increase compared to wild-type controls probably as a result of differences in strain background
|
• at 10 and 14 months of age
|
• impaired performance in a balance beam test at 10 and 14 months of age
|
• at 10 and 14 months of age compared to mutant mice not carrying the transgene
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
Alzheimer's disease | DOID:10652 | J:128106 |
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO) |
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last database update 12/10/2024 MGI 6.24 |
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