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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(PRNP-APPSweInd)8Dwst
transgene insertion 8, David Westaway
MGI:3589475
Summary 18 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cx1
Azdm1A/J/Azdm1A/J
Tg(PRNP-APPSweInd)8Dwst/0
involves: A/J * C57BL/6J MGI:3818007
cx2
Azdm2A/J/Azdm2A/J
Tg(PRNP-APPSweInd)8Dwst/0
involves: A/J * C57BL/6J MGI:3818010
cx3
Azdm3A/J/Azdm3C57BL/6J
Tg(PRNP-APPSweInd)8Dwst/0
involves: A/J * C57BL/6J MGI:3818011
cx4
Azdm3A/J/Azdm3A/J
Tg(PRNP-APPSweInd)8Dwst/0
involves: A/J * C57BL/6J MGI:3818012
cx5
Azdm4A/J/Azdm4C57BL/6J
Tg(PRNP-APPSweInd)8Dwst/0
involves: A/J * C57BL/6J MGI:3818013
cx6
Azdm4A/J/Azdm4A/J
Tg(PRNP-APPSweInd)8Dwst/0
involves: A/J * C57BL/6J MGI:3818014
cx7
Azdm1A/J/Azdm1C57BL/6J
Tg(PRNP-APPSweInd)8Dwst/0
involves: A/J * C57BL/6J MGI:3818008
cx8
Azdm2A/J/Azdm2C57BL/6J
Tg(PRNP-APPSweInd)8Dwst/0
involves: A/J * C57BL/6J MGI:3818009
cx9
Npc1m1N/Npc1m1N
Tg(PRNP-APPSweInd)8Dwst/0
involves: BALB/c * C3H/HeJ * C57BL/6J MGI:5442379
cx10
Atp7btx-J/Atp7btx-J
Tg(PRNP-APPSweInd)8Dwst/0
involves: C3H/HeJ * C57BL/6 MGI:3793275
cx11
Tg(PRNP-APPSweInd)8Dwst/0
Tg(PSEN1)1098Citr/0
involves: C3H/HeJ * C57BL/6J * FVB/N MGI:3722166
cx12
Tg(Camk2a-ITM2B)8.4Ldad/0
Tg(PRNP-APPSweInd)8Dwst/0
involves: C3H/HeJ * C57BL/6J * FVB/N MGI:3810992
cx13
Tg(PRNP-APPSweInd)8Dwst/0
Tg(PSEN1*L286V)1274Citr/0
involves: C3H/HeJ * C57BL/6J * FVB/N MGI:3722165
tg14
Tg(PRNP-APPSweInd)8Dwst/0 involves: 129S6/SvEvTac * C3H/HeJ * C57BL/6J MGI:3722163
tg15
Tg(PRNP-APPSweInd)8Dwst/0 involves: C3H/HeJ * C57BL/6 MGI:3793276
tg16
Tg(PRNP-APPSweInd)8Dwst/0 involves: C3H/HeJ * C57BL/6J MGI:3722160
tg17
Tg(PRNP-APPSweInd)8Dwst/0 involves: C3H/HeJ * C57BL/6J * FVB/N MGI:3722161
tg18
Tg(PRNP-APPSweInd)8Dwst/? involves: 129S6/SvEvTac MGI:3665287


Genotype
MGI:3818007
cx1
Allelic
Composition
Azdm1A/J/Azdm1A/J
Tg(PRNP-APPSweInd)8Dwst/0
Genetic
Background
involves: A/J * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• decreased brain amyloid-beta 40 and 42

homeostasis/metabolism
• decreased brain amyloid burden
• decreased brain amyloid plaque number
• decreased brain amyloid-beta 40 and 42




Genotype
MGI:3818010
cx2
Allelic
Composition
Azdm2A/J/Azdm2A/J
Tg(PRNP-APPSweInd)8Dwst/0
Genetic
Background
involves: A/J * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• decreased brain amyloid burden
• decreased brain amyloid plaque number




Genotype
MGI:3818011
cx3
Allelic
Composition
Azdm3A/J/Azdm3C57BL/6J
Tg(PRNP-APPSweInd)8Dwst/0
Genetic
Background
involves: A/J * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• decreased brain amyloid burden
• decreased brain amyloid plaque number




Genotype
MGI:3818012
cx4
Allelic
Composition
Azdm3A/J/Azdm3A/J
Tg(PRNP-APPSweInd)8Dwst/0
Genetic
Background
involves: A/J * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• decreased brain amyloid burden
• decreased brain amyloid plaque number




Genotype
MGI:3818013
cx5
Allelic
Composition
Azdm4A/J/Azdm4C57BL/6J
Tg(PRNP-APPSweInd)8Dwst/0
Genetic
Background
involves: A/J * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• decreased brain amyloid plaque number




Genotype
MGI:3818014
cx6
Allelic
Composition
Azdm4A/J/Azdm4A/J
Tg(PRNP-APPSweInd)8Dwst/0
Genetic
Background
involves: A/J * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• decreased brain amyloid plaque number




Genotype
MGI:3818008
cx7
Allelic
Composition
Azdm1A/J/Azdm1C57BL/6J
Tg(PRNP-APPSweInd)8Dwst/0
Genetic
Background
involves: A/J * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• decreased brain amyloid-beta 40 and 42

homeostasis/metabolism
• decreased brain amyloid burden
• decreased brain amyloid plaque number
• decreased brain amyloid-beta 40 and 42




Genotype
MGI:3818009
cx8
Allelic
Composition
Azdm2A/J/Azdm2C57BL/6J
Tg(PRNP-APPSweInd)8Dwst/0
Genetic
Background
involves: A/J * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• decreased brain amyloid burden
• decreased brain amyloid plaque number




Genotype
MGI:5442379
cx9
Allelic
Composition
Npc1m1N/Npc1m1N
Tg(PRNP-APPSweInd)8Dwst/0
Genetic
Background
involves: BALB/c * C3H/HeJ * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Npc1m1N mutation (3 available); any Npc1 mutation (74 available)
Tg(PRNP-APPSweInd)8Dwst mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mutants have a maximum lifespan of 77 days, with mortality rate increasing drastically from 55 days onward
• mutants treated with 2-hydroxypropyl-beta-cyclodextrin (2-HPC) at P7 to lower cholesterol accumulation live significantly longer than saline-injected mutants, with a median survival of 103 days versus 69 days

growth/size/body
• mutants reach a maximum body weight of approximately 12 grams by 6 weeks of age which is about 30% less compared to controls
• weight declines progressively after 6 weeks of age until death

behavior/neurological
• mutants exhibit deficits in object memory index at 7 and 10 weeks of age; object recognition memory deficits are accelerated compared to single Npc1 homozygotes
• mutants treated with 2-HPC at P7 to lower cholesterol accumulation show significant improvement in cognitive performance
• mutants exhibit impaired rotarod performance and gait coordination at 7 weeks of age which is further exacerbated by 10 weeks of age
• mutants treated with 2-HPC at P7 to lower cholesterol accumulation show significant improvement in motor performance
• mutants exhibit reduced locomotor activity and increased periods of inactivity in open-field tests at both 7 and 10 weeks of age
• mutants treated with 2-HPC at P7 to lower cholesterol accumulation show significant improvement in motor performance

nervous system
• mutants exhibit microglial activation in the hippocampus and cerebellum at a higher level than in single Npc1 homozygotes
• mutants treated with 2-HPC at P7 to lower cholesterol accumulation show lower microglia activation compared to saline-injected mutants
• mutants exhibit intracellular accumulation of unesterified cholesterol in the hippocampus and cerebellum at 4, 7, and 10 weeks of age
• however total cholesterol content in the hippocampus and cerebellum is not altered compared with controls
• mutants exhibit a profound increase in the number and activation of glial fibrillary acidic protein (GFAP)-labeled astrocytes in the hippocampus and cerebellum compared with wild-type, single Tg(PRNP-APPSweInd)8Dwst mutants, and single Npc1 homozygotes
• mutants treated with 2-HPC at P7 to lower cholesterol accumulation show decreased astrocyte activation compared to saline-injected mutants
• neurodgeneration in the cerebellum is accelerated compared to single Npc1 homozygotes
• decrease in the number of cerebellar Purkinje cells that is more pronounced than in single Npc1 homozygotes
• however neuronal loss in the hippocampus is not seen
• mutants exhibit a loss of myelin fibre tracts in the hippocampus/cortex and cerebellum, indicating significant demyelination; demyelination is more severe than in single Npc1 homozygotes

hematopoietic system
• mutants exhibit microglial activation in the hippocampus and cerebellum at a higher level than in single Npc1 homozygotes
• mutants treated with 2-HPC at P7 to lower cholesterol accumulation show lower microglia activation compared to saline-injected mutants

homeostasis/metabolism
• cathepsin D levels are higher in the hippocampus and the cerebellum than in controls, including single Npc1 homozygotes
• cytosolic cathepsin D, cytochrome c and Bcl-2-associated X protein levels are increased in the cerebellum to a higher level than in single Npc1 homozygotes
• mutants exhibit intracellular accumulation of unesterified cholesterol in the hippocampus and cerebellum at 4, 7, and 10 weeks of age
• however total cholesterol content in the hippocampus and cerebellum is not altered compared with controls
• cathepsin D activity is higher in the hippocampus and the cerebellum than in controls, including single Npc1 homozygotes

immune system
• mutants exhibit microglial activation in the hippocampus and cerebellum at a higher level than in single Npc1 homozygotes
• mutants treated with 2-HPC at P7 to lower cholesterol accumulation show lower microglia activation compared to saline-injected mutants

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Alzheimer's disease DOID:10652 J:188345
Niemann-Pick disease DOID:14504 J:188345




Genotype
MGI:3793275
cx10
Allelic
Composition
Atp7btx-J/Atp7btx-J
Tg(PRNP-APPSweInd)8Dwst/0
Genetic
Background
involves: C3H/HeJ * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Atp7btx-J mutation (1 available); any Atp7b mutation (82 available)
Tg(PRNP-APPSweInd)8Dwst mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
N
• survival is increased compared to in Tg(PRNP-APPSweInd)8Dwst mice

homeostasis/metabolism
• mice exhibit a 45% reduction in plaque area compared to in Tg(PRNP-APPSweInd)8Dwst mice due to a reduction in the number of plaques
• copper levels are increased compared to in heterozygous Atp7btx-J mice

nervous system
• mice exhibit a 45% reduction in plaque area compared to in Tg(PRNP-APPSweInd)8Dwst mice due to a reduction in the number of plaques




Genotype
MGI:3722166
cx11
Allelic
Composition
Tg(PRNP-APPSweInd)8Dwst/0
Tg(PSEN1)1098Citr/0
Genetic
Background
involves: C3H/HeJ * C57BL/6J * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• amyloid deposition is enhanced when the PSEN1 transgene is also expressed by mutants; deposition is noted by 30-45 days of age




Genotype
MGI:3810992
cx12
Allelic
Composition
Tg(Camk2a-ITM2B)8.4Ldad/0
Tg(PRNP-APPSweInd)8Dwst/0
Genetic
Background
involves: C3H/HeJ * C57BL/6J * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• reduction in plaque formation compared to mice carrying just Tg(PRNP-APPSweInd)8Dwst

homeostasis/metabolism
• reduction in plaque formation compared to mice carrying just Tg(PRNP-APPSweInd)8Dwst




Genotype
MGI:3722165
cx13
Allelic
Composition
Tg(PRNP-APPSweInd)8Dwst/0
Tg(PSEN1*L286V)1274Citr/0
Genetic
Background
involves: C3H/HeJ * C57BL/6J * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• amyloid deposition is enhanced when the PSEN1 transgene is also expressed by mutants; deposition is noted by 30-45 days of age




Genotype
MGI:3722163
tg14
Allelic
Composition
Tg(PRNP-APPSweInd)8Dwst/0
Genetic
Background
involves: 129S6/SvEvTac * C3H/HeJ * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• Background Sensitivity: survival decreases to 25-40% within 120 days of life; only 17% (7/41) survive to 365 days




Genotype
MGI:3793276
tg15
Allelic
Composition
Tg(PRNP-APPSweInd)8Dwst/0
Genetic
Background
involves: C3H/HeJ * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• copper levels are decreased compared to in non-transgenic mice




Genotype
MGI:3722160
tg16
Allelic
Composition
Tg(PRNP-APPSweInd)8Dwst/0
Genetic
Background
involves: C3H/HeJ * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype

Increase in creatine deposits in hippocampus of Tg(PRNP-APPSweInd)8Dwst/0 mice with age

mortality/aging
• Background Sensitivity: 20/52 mice die before 120 days, 3/52 by 130 days and 3/52 die after 250 days; 50% survive for at least a year

behavior/neurological
• mice show impairment in acquisition of spatial information during place discrimination training at 11 weeks of age; mice show longer latencies to reach escape platform and longer search paths to reach platform
• during probe trial after completion of training, mice display lower annulus crossing index, searching for platform in a circular fashion and often crossing the centers of alternative quadrants, relative to non-transgenic controls

nervous system
N
• no differences are seen in volume of dorsal hippocampus or in neuronal cytoarchitecture between transgenic and control mice
• dense-cored amyloid deposits contain amyloid beta-42 peptide (J:69967)
• 14-month old mutants show dense amyloid beta core plaques in the hippocampus (J:166801)
• by 101 days, plaques are detected within the pial vessels, and in the cerebral vasculature by 196 days
• amyloid plaques are associated with dystrophic neurites; dystrophic neuron pathology increases with age and frequency of large cored plaques

homeostasis/metabolism
• potent deposition of cerebral amyloid is observed in all mice 90 days of age
• dense-cored amyloid deposits contain amyloid beta-42 peptide (J:69967)
• 14-month old mutants show dense amyloid beta core plaques in the hippocampus (J:166801)
• by 101 days, plaques are detected within the pial vessels, and in the cerebral vasculature by 196 days
• increase in creatine deposits in the hippocampal gray matter as mutants age

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Alzheimer's disease DOID:10652 J:166801




Genotype
MGI:3722161
tg17
Allelic
Composition
Tg(PRNP-APPSweInd)8Dwst/0
Genetic
Background
involves: C3H/HeJ * C57BL/6J * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• Background Sensitivity: survival decreases to 25-40% within 120 days of life; only 3/12 mice survive to 365 days
• survival is less than on other genetic backgrounds examined




Genotype
MGI:3665287
tg18
Allelic
Composition
Tg(PRNP-APPSweInd)8Dwst/?
Genetic
Background
involves: 129S6/SvEvTac
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• observed at 16 and 20 weeks of age in hippocampus and cortex
• level of brain amyloid beta peptide 42 is predominant over 40; levels increase dramatically after 16 weeks of age

homeostasis/metabolism
• level of brain amyloid beta peptide 42 is predominant over 40; levels increase dramatically after 16 weeks of age
• observed at 16 and 20 weeks of age in hippocampus and cortex
• elevated plasma triglyceride levels observed between 9 and 15 weeks of age
• VLDL production rates are increased as early as 9 weeks of age and remain elevated up to 22 weeks of age
• plasma free fatty acid levels were unchanged

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Alzheimer's disease DOID:10652 J:113199





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last database update
10/29/2024
MGI 6.24
The Jackson Laboratory