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340031 RESULTS

PSEN1 Knock-out

RESEARCH MODELS Summary These mice are deficient in PSEN1. This model is perinatal lethal in homozygouse animals which die shortly after birth, presumably from breathing difficulties due to ribcage deformity (Shen et al., 1997). Gross skeletal malformations and central n

APP(V717I)

RESEARCH MODELS Modification Details Transgene containing human APP (isoform 695) with the London mutation driven by the Thy1 promoter. Neuropathology Plaques start in the subiculum at ten months, spreading to the frontal cortex as dense and diffuse aggregates. Frequent

Tau P301L

RESEARCH MODELS Neuropathology Tau hyperphosphorylation and conformational changes in the brain parenchyma start around seven months. Tangle-like pathology is mainly observed in the brain stem and spinal cord, and to a lesser extent in the midbrain and cerebral cortex (T

APP(V717I) x PS1(A246E)

RESEARCH MODELS Summary These are bigenic mice that overexpress both mutant APP (V717I mutation) and mutant PSEN1 (A246E mutation). They were first generated and described by the laboratory of Fred Van Leuven (Dewachter et al., 2000). The transgenes are coexpressed in th

Senescence Accelerated Mouse (SAMP8)

RESEARCH MODELS Summary The Senescence Accelerated Mouse-Prone 8 (SAMP8) is a naturally occuring mouse line that displays a phenotype of accelerated aging. While maintaining an inbred AKR/J line in the early 1970's, researchers at Kyoto University became aware that

Progranulin

ALZPEDIA Progranulin burst on the scene of neurodegenerative disease as a major genetic cause of frontotemporal dementia (FTD) in 2006, only months before TDP-43 was identified as the main protein constituent of the histopathological lesions in the same patients.

Tau Seed Detection via FRET Flow Cytometry

PROTOCOL This assay was designed to detect small amounts of tau seeds in biological samples, such as brain homogenates from humans or rodent tauopathy models. HEK-293T cells stably expressing a human tau sequence were engineered to serve as biosensors of intracell

APPSwe (line C3-3)

RESEARCH MODELS Summary These transgenic mice express a chimeric mouse/human APP carrying the Swedish mutation under the control of the mouse prion protein promoter. This line, C3-3, was generated in parallel with line E1-2, which also expresses APP carrying the Swedish

PS1(M146L)

RESEARCH MODELS Summary The first report of this transgenic line described two lines of mice that overexpress human PSEN1 with the M146L mutation, line 5.1 and line 6.2 (Duff et al., 1996). Line 5.1 has been more extensively characterized, especially in relation to the d

SORLA (SORL1)

ALZPEDIA The sortilin-related receptor SORLA is an endocytic receptor that belongs to the vacuolar protein sorting 10 (VPS10) domain receptor family. SORLA binds to the amyloid precursor protein (APP). It functions as an intracellular sorting receptor as APP is be

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