Phenotypes associated with this allele
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Psen1tm1Lpr mutation
(0 available);
any
Psen1 mutation
(46 available)
Tg(Thy1-APPSL)28Lpr mutation
(0 available)
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nervous system
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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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• 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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homeostasis/metabolism
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Psen1tm1Lpr mutation
(0 available);
any
Psen1 mutation
(46 available)
Tg(Thy1-APPSL)28Lpr mutation
(0 available)
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nervous system
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• 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)
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• 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
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• 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
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• decrease in the number of dentate gyrus granule cells at 12 months of age
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• 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
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• thickened and irregularly shaped neurites are seen in the medulla at 14 months of age
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• 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
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• 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
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homeostasis/metabolism
behavior/neurological
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• at 10 and 14 months of age
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• impaired performance in a balance beam test at 10 and 14 months of age
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growth/size/body
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• at 10 and 14 months of age compared to mutant mice not carrying the transgene
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
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nervous system
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• plaques are seen at 6 months of age
(J:86694)
• detected in the hippocampus at 4.5 and 17 months of age
(J:99507)
• abundant amyloid beta neuropil deposits in hippocampal and cortical areas in aged mice
(J:128243)
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• non-corrected intracranial and brain volumes are reduced compared to Tg(Hmgcr-PSEN1*M146L)#Lpr single transgenics at 10 weeks of age
• corrected brain volumes are strongly decreased with age
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• in aged mice compared to Tg(Hmgcr-PSEN1*M146L)#Lpr single transgenics
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• significant increase in brain length with age compared to Tg(Hmgcr-PSEN1*M146L)#Lpr single transgenics
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• surface area of the internal capsule does not increase with age unlike in Tg(Hmgcr-PSEN1*M146L)#Lpr single transgenics
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• decrease in thickness of the caudal corpus callosum with age
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• decrease in fornix diameter in aged mice
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• age-related decline in mean synaptic bouton densities within the stratum lucidum of area CA3
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• age-related decline in mean synaptic bouton densities and in mean volume
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• age-related decline in mean synaptic bouton densities in area CA1?2
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• clusters of swollen and abnormally distorted neuritic profiles are seen
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• age-related decline in mean synaptic bouton densities within the hippocampal molecular cell layer, stratum lucidum of area CA3, and stratum radiatum of area CA1?2
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• global brain atrophy in aged mice
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homeostasis/metabolism
growth/size/body
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• brain weight to body weight ratio is increased compared to Tg(Hmgcr-PSEN1*M146L)#Lpr single transgenics at 24 months of age
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• in aged mice compared to Tg(Hmgcr-PSEN1*M146L)#Lpr single transgenics
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
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mortality/aging
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• enhanced lethality at young ages (less than 4 months of age)
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behavior/neurological
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• spend more time in the closed arms of an elevated plus maze at 15 months of age compared to age matched controls
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• cross fewer segments and show a reduced latency to fall in a stationary beam test at 15 months of age
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• significant decrease in latency to fall in a wire grid assay at 3 and 15, but not at 6, months of age
• significant decrease in latency to fall in a coat hanger assay at 15 months of age
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• at 3, 6, and 15 months of age in an open field test
• activity decreases with age
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nervous system
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• increase in brain length but not weight
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growth/size/body
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• from 3 to 15 months of age
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homeostasis/metabolism
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
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nervous system
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• plaques are seen at 6 months of age
(J:86694)
• detected in the hippocampus at 17 months of age
(J:99507)
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• levels of SDS-soluble amyloid beta protein are higher in females than in males at 3 months of age
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• age-related decline in mean synaptic bouton densities
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• age-related decline in mean synaptic bouton densities in area CA1?2
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• clusters of swollen and abnormally distorted neuritic profiles are seen
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• age-related decline in mean synaptic bouton densities within the hippocampal molecular cell layer and stratum radiatum of area CA1?2
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• elevated levels of lipid peroxidation products in brain homogenates in females at 3 months of age and in males and females at 12 months of age
• reduced levels of antioxidant enzymes at 3 and 12 months of age
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• impaired mitochondrial function
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cellular
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• mitochondrial membrane potentials and ATP levels are reduced in dissociated brain cells at 3 months of age
• inhibition of complex IV and significantly reduced cytochrome c oxidase activity in cerebral isolated mitochondria
• state 3 and state 4 respiration are significantly reduced in freshly isolated cerebral mitochondria at 17 months of age
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homeostasis/metabolism