Species: Rat
Genes: APP, PSEN1
Modification: APP: Transgenic; PSEN1: Transgenic
Disease Relevance: Alzheimer's Disease
Strain Name: N/A
Summary
APP+PS1 transgenic rats express human APP with the Swedish and Indiana mutations and human PSEN1 with the L166P mutation. Both transgenes are driven by the ubiquitin-C promoter. These rats exhibit amyloid pathology, neurodegeneration, and behavioral deficits.
Phenotype Characterization
When visualized, these models will distributed over a 18 month
timeline
demarcated at the following intervals: 1mo, 3mo, 6mo,
9mo, 12mo, 15mo, 18mo+.
Plaques
Abundant plaques in hippocampus and subiculum, scattered plaques in cortex.
Neuronal Loss
Necrotic neurons in hippocampus and cortex.
Cognitive Impairment
Deficits in Barnes maze at 10 months.
Last Updated: 31 Aug 2018
Further Reading
No Available Further Reading
Species: Rat
Genes: APP
Modification: APP: Transgenic
Disease Relevance: Alzheimer's Disease
Strain Name: F344-Tg(APP)21Besey
Summary
Phenotype Characterization
When visualized, these models will distributed over a 18 month
timeline
demarcated at the following intervals: 1mo, 3mo, 6mo,
9mo, 12mo, 15mo, 18mo+.
Plaques
Do not spontaneously develop amyloid pathology, but can serve as hosts for exogenously seeded amyloid deposits.
Tangles
“Flame-shaped” profiles in hippocampal neurons of 18- to 19-month-old female rats revealed by hematoxylin-and-eosin-staining.
Neuronal Loss
Necrotic neurons in hippocampus and cortex of female rats.
Gliosis
Activated (MHCII-positive) microglia present in white matter tracts at 15 months.
Cognitive Impairment
Male rats show deficits in Morris water maze as early as 3 months of age. Females show deficits in Barnes maze at 14 months of age.
Last Updated: 31 Aug 2018
Further Reading
No Available Further Reading
Species: Mouse
Genes: Tardbp
Mutations: Tardp Q331K
Modification: Tardbp: Knock-In
Disease Relevance: Frontotemporal Dementia, Amyotrophic Lateral Sclerosis
Strain Name: N/A
Summary
CRISPR/Cas9 was used to introduce the p.Q331K mutation into the mouse Tardp gene (Fratta et al., 2018). These mice can be used to study the effects of the p.Q331K mutation when TDP-43 is expressed at physiological levels, under the control of its natural regulatory elements.
Phenotype Characterization
When visualized, these models will distributed over a 18 month
timeline
demarcated at the following intervals: 1mo, 3mo, 6mo,
9mo, 12mo, 15mo, 18mo+.
Last Updated: 17 Aug 2018
Further Reading
No Available Further Reading
Species: Mouse
Genes: Tardbp
Mutations: Tardp F210I
Modification: Tardbp: Other
Disease Relevance: Frontotemporal Dementia, Amyotrophic Lateral Sclerosis
Strain Name: Tardbp F210I
Summary
Phenotype Characterization
When visualized, these models will distributed over a 18 month
timeline
demarcated at the following intervals: 1mo, 3mo, 6mo,
9mo, 12mo, 15mo, 18mo+.
Premature Death
Homozygous mutation is embryonic lethal.
Last Updated: 17 Aug 2018
Further Reading
No Available Further Reading
Species: Mouse
Genes: Tardbp
Mutations: Tardbp M323K
Modification: Tardbp: Other
Disease Relevance: Frontotemporal Dementia, Amyotrophic Lateral Sclerosis
Strain Name: Tardbp M323K
Summary
Phenotype Characterization
When visualized, these models will distributed over a 18 month
timeline
demarcated at the following intervals: 1mo, 3mo, 6mo,
9mo, 12mo, 15mo, 18mo+.
Lower Motor Neuron Loss
28 percent reduction in the number of motor neurons in the sciatic motor neuron pool, compared with non-transgenic littermates.
Cytoplasmic Inclusions
At 18 months of age, p62- and ubiquitin-positive inclusions are found in the ventral regions of the spinal cord, although these inclusions do not appear to be located in the cytoplasm of motor neurons.
NMJ Abnormalities
By 24 months, a 15 percent reduction in motor units innervating the extensor digitorum muscle.
Motor Impairment
Grip strength in both male and female mice begins to decline at 12 months of age. By 24 months, there is a nearly 40 percent reduction in force measured in tibialis anterior muscles.
Premature Death
Do not exhibit premature death, at least until 24 months of age.
Last Updated: 17 Aug 2018
Further Reading
No Available Further Reading
Species: Mouse
Genes: Trem2, APP, PSEN1
Modification: Trem2: Knock-In; APP: Transgenic; PSEN1: Transgenic
Disease Relevance: Alzheimer's Disease
Strain Name: N/A
Summary
Phenotype Characterization
When visualized, these models will distributed over a 18 month
timeline
demarcated at the following intervals: 1mo, 3mo, 6mo,
9mo, 12mo, 15mo, 18mo+.
Plaques
Reduction in the number and burden of fibrillar amyloid plaques in the hippocampus, compared with APPPS1-21 mice homozygous for wild-type Trem2.
Tangles
Tangles were not observed at 4 months of age, but hyperphosphorylated tau was detected in dystrophic neurites surrounding plaques.
Neuronal Loss
No differences in neuron number in cotical layer V in APPPS1-21;Trem2+/R47H mice relative to APPPS1-21 mice homozygous for wild-type Trem2, at 4 months of age.
Gliosis
Fewer plaque-associated myeloid cells in APPPS1-21;Trem2+/R47H, compared with APPPS1-21 mice homozygous for wild-type Trem2.
Last Updated: 03 Aug 2018
Further Reading
No Available Further Reading
Species: Mouse
Genes: Trem2
Modification: Trem2: Knock-In
Disease Relevance: Alzheimer's Disease
Strain Name: N/A
Summary
Phenotype Characterization
When visualized, these models will distributed over a 18 month
timeline
demarcated at the following intervals: 1mo, 3mo, 6mo,
9mo, 12mo, 15mo, 18mo+.
Plaques
No 6E10- or Thioflavin S-positive amyloid plaques were observed at 4 months of age.
Last Updated: 05 Aug 2018
Further Reading
No Available Further Reading
Species: Mouse
Genes: APOE, App, Trem2
Modification: APOE: Knock-In; App: Knock-Out; Trem2: Knock-In
Disease Relevance: Alzheimer's Disease
Strain Name: B6.Cg-Apoetm1.1(APOE*4)Adiuj Appem2Adiuj Trem2em1Adiuj/J
Summary
The epsilon-4 allele of Apoliporotein E (APOE4) and the R47H variant of TREM2 have each been found to confer an approximately threefold increased risk for Alzheimer’s disease in humans heterozygous for either allele. This triple mutant line carries a humanized APOE4 gene, the p.R47H mutation knocked into mouse Trem2, and a 94-bp deletion in exon 14 (APP695 numbering) of the mouse App gene. This line may be useful for studying late-onset, sporadic Alzheimer's disease.
Phenotype Characterization
When visualized, these models will distributed over a 18 month
timeline
demarcated at the following intervals: 1mo, 3mo, 6mo,
9mo, 12mo, 15mo, 18mo+.
Last Updated: 30 Nov 2018
Further Reading
No Available Further Reading
Species: Mouse
Genes: APP
Modification: APP: Knock-In
Disease Relevance: Alzheimer's Disease
Strain Name: B6(SJL)-Apptm1.1Aduci/J
Summary
These mice express APP with a “humanized” Aβ sequence. Three point mutations were introduced into exon 14 (exon numbering according to APP695, with 16 exons) of the mouse App gene, to produce three amino acid substitutions within the Aβ sequence (amino acids 5 (G to R), 10 (F to Y) and 13 (R to H) of Aβ). Exon 14 is also flanked by loxP sites. The human Aβ generated by these mice is expected to be more aggregation-prone than the endogenous mouse Aβ. This line may be useful for studying late-onset sporadic Alzheimer’s disease.
Phenotype Characterization
When visualized, these models will distributed over a 18 month
timeline
demarcated at the following intervals: 1mo, 3mo, 6mo,
9mo, 12mo, 15mo, 18mo+.
Last Updated: 09 Apr 2019
Further Reading
No Available Further Reading
Species: Mouse
Genes: APOE, Plcg2, Trem2
Modification: APOE: Knock-In; Plcg2: Knock-In; Trem2: Knock-In
Disease Relevance: Alzheimer's Disease
Strain Name: B6.Cg-Apoetm1.1(APOE*4)Adiuj Plcg2em2Adiuj Trem2em1Adiuj/J
Summary
Phenotype Characterization
When visualized, these models will distributed over a 18 month
timeline
demarcated at the following intervals: 1mo, 3mo, 6mo,
9mo, 12mo, 15mo, 18mo+.
Last Updated: 30 Nov 2018
Further Reading
No Available Further Reading
COMMENTS / QUESTIONS
No Available Comments
Make a comment or submit a question
To make a comment you must login or register.