
JNPL3(P301L) - ALZFORUM
2025年1月24日 · These mice carry a transgene encoding human tau with four microtubule-binding repeat domains and no N-terminal inserts (4R/0N). The transgene contains the P301L mutation and expression is driven by the mouse prion promoter.
Mouse Models of Alzheimer’s Disease - PMC - PubMed Central …
JNPL3 Mice. JNPL3 mice were the first mouse model established to carry a MAPT mutation (Lewis et al., 2000). They express human 0N4R tau with the P301L mutation, driven by the mouse prion protein promoter.
Gait Ignition Failure in JNPL3 Human Tau-mutant Mice - PMC
In JNPL3 transgenic (Tg) mice, expression of human tau containing the most common FTDP-17 mutation (P301L) under the control of the mouse prion promoter resulted in motor disturbances including weakened grasping strength, hunched posture, and hindlimb paralysis with age- and gene-dose-dependent development of NFT [11].
Neurofibrillary tangles, amyotrophy and progressive motor …
2000年9月1日 · We analysed sarkosyl-insoluble tau from brains and spinal cords of JNPL3 mice at progressive ages, littermate controls, JN4 and JN25 (WT4R) mice by western blot using human tau-specific...
Enhanced neurofibrillary degeneration in transgenic mice …
2001年8月24日 · JNPL3 transgenic mice expressing a mutant tau protein, which develop neurofibrillary tangles and progressive motor disturbance, were crossed with Tg2576 transgenic mice expressing mutant beta-amyloid precursor protein (APP), thus modulating the APP-Abeta (beta-amyloid peptide) environment.
Neuronal hypofunction and network dysfunction in a mouse …
2024年10月5日 · A large fraction of neurons were hypoactive in JNPL3 mice. Treadmill running revealed that JNPL3 mice had less activated and more suppressed neurons compared to WT mice. Despite fewer activated neurons in JNPL3 mice, higher synchrony of Ca 2+ activity was observed in resting animals, and disrupted engagement of neuronal Ca 2+ activity during ...
Small molecule inhibitor of tau self-association in a mouse
2023年8月9日 · The P301L mutation makes tau prone to aggregation; therefore, JNPL3 mice present a more challenging target than mouse models of human tau without mutations. JNPL3 mice were treated from 3 to 7 months of age with Vehicle, 30 mg/kg compound dose, or 40 mg/kg compound dose.
Enhanced Neurofibrillary Degeneration in Transgenic Mice
2001年8月24日 · Hemizygous JNPL3 mice develop progressive motor and behavioral abnormalities with robust neurofibrillary pathology and neuronal loss in the spinal cord as early as 6.5 months (20). In JNPL3 animals, NFTs are primarily located in the spinal cord and the hindbrain, with fewer NFTs in the midbrain, amygdala, and hypothalamus (20).
Gait Ignition Failure in JNPL3 Human Tau-mutant Mice
Accordingly, immunohistochemistry revealed highly phosphorylated paired helical filament tau in JNPL3 brainstem areas associated with gait initiation. Together, these findings demonstrate a novel behavioral phenotype of impaired gait initiation in JNPL3 mice and underscore the value of this mouse line as a tool to study the neural mechanisms ...
Gene expression profiling of the tau mutant (P301L) transgenic mouse ...
2001年9月7日 · In this study, to examine the transcriptional profiles associated with FTDP-17 Tau mutation in brain, we used a transgenic mouse line, JNPL3, expressing human Tau with the P301L mutation that develops NFT with neuronal loss [13].