Tauopathies
|
|
0.100 |
GeneticVariation
|
BEFREE |
Because the mutations (V337M, P301L) are associated with genetic tauopathies, these results suggest that a factor in disease etiology of genetic tauopathies and other dementias with altered tau is a greater abundance of tau in the cytoplasm due to decreased binding to microtubules.
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11170176 |
2001 |
Tauopathies
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0.100 |
GeneticVariation
|
BEFREE |
Together, our results show that expression of the P301L mutation in mice causes neuronal lesions that are similar to those seen in human tauopathies.
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11013246 |
2001 |
Tauopathies
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0.100 |
GeneticVariation
|
BEFREE |
P301L tauopathy: confocal immunofluorescence study of perinuclear aggregation of the mutated protein.
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12127682 |
2002 |
Tauopathies
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|
0.100 |
GeneticVariation
|
BEFREE |
The tauopathy in P301L and G272V does not appear to be associated with an evident increase in CSF levels of Ptau-181 in FTD patients with these tau mutations, in contrast with findings in patients with AD.
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12975285 |
2003 |
Tauopathies
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0.100 |
GeneticVariation
|
BEFREE |
Lastly, we have demonstrated that tau is phosphorylated on Tyr-18 in the tau P301L mouse model for tauopathy (JNPL3).
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16115884 |
2005 |
Tauopathies
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0.100 |
GeneticVariation
|
BEFREE |
Thus, Pin1 has opposite effects on the tauopathy</span> p</span>henotype depending on whether the tau is WT or a P301L mu</span>tant, indicating the need for disease-specific therapies for tauopathies.
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18431510 |
2008 |
Tauopathies
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0.100 |
GeneticVariation
|
BEFREE |
In the present study, we employed a somatic cell gene transfer technique to create a rodent model of tauopathy by injecting a recombinant adeno-associated viral vector with a mutated human tau gene (P301L) into the hippocampus of adult rats.
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22561128 |
2012 |
Tauopathies
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0.100 |
GeneticVariation
|
BEFREE |
The JNPL3 mice express human tau proteins bearing a P301L mutation, which mimics the neurodegenerative process observed in humans with tauopathy.
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22975846 |
2012 |
Tauopathies
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0.100 |
GeneticVariation
|
BEFREE |
Here we used the non-invasive, Manganese-Enhanced Magnetic Resonance Imaging technique (MEMRI), to study for the first time a pure model of tauopathy, the JNPL3 transgenic mouse line, which overexpresses a mutated (P301L) form of the human tau protein.
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22960250 |
2013 |
Tauopathies
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0.100 |
GeneticVariation
|
BEFREE |
Immunophilin FKBP52 induces Tau-P301L filamentous assembly in vitro and modulates its activity in a model of tauopathy.
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24623856 |
2014 |
Tauopathies
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0.100 |
GeneticVariation
|
BEFREE |
In addition to classic markers of tauopathy, significant neuroinflammation and extensive gliosis were detected in AAV1-Tau(P301L) mice.
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26276810 |
2015 |
Tauopathies
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0.100 |
GeneticVariation
|
BEFREE |
Here, we found that the BDNF level was reduced in the serum and brain of AD patients and P301L transgenic mice (a mouse model of tauopathy).
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27701410 |
2016 |
Tauopathies
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0.100 |
GeneticVariation
|
BEFREE |
Clinicopathologic heterogeneity in frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17) due to microtubule-associated protein tau (MAPT) p.P301L mutation, including a patient with globular glial tauopathy.
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27859539 |
2017 |
Tauopathies
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0.100 |
GeneticVariation
|
BEFREE |
Neuroprotective effects of low fat-protein diet in the P301L mouse model of tauopathy.
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28456717 |
2017 |
Tauopathies
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0.100 |
GeneticVariation
|
BEFREE |
The TauP301L mouse expresses P301L tau under the control of a prion promoter in both neurons and astrocytes, reminiscent of some human tauopathies.
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28869476 |
2017 |
Tauopathies
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|
0.100 |
GeneticVariation
|
BEFREE |
In this study, we found that aged Tg mice of both sexes expressing human tau proteins harboring a pathogenic P301L <i>MAPT</i> mutation labeled with green fluorescent protein (T40PL-GFP Tg mouse line) exhibited hyperphosphorylated tau mislocalized to the somatodentritic domain of neurons, but these mice did not develop <i>de novo</i> insoluble tau aggregates, which are characteristic of human AD and related tauopathies.
|
28986461 |
2017 |
Tauopathies
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|
0.100 |
GeneticVariation
|
BEFREE |
We evaluated two structurally similar natural compounds, morin and resveratrol, for treating tauopathy in JNPL3 P301L mutant human tau overexpressing mice.
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30479844 |
2018 |
Tauopathies
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|
0.100 |
GeneticVariation
|
BEFREE |
Interestingly, FTLD-tau cases with MAPT mutations had similar patterns and severity of neuropathological features to sporadic FTLD-tau subtypes and could be classified into: Pick's disease (K257T), corticobasal degeneration (S305S, IVS10+16, R406W), progressive supranuclear palsy (S305S) or globular glial tauopathy (P301L, IVS10+16).
|
29253099 |
2018 |
Tauopathies
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0.100 |
GeneticVariation
|
BEFREE |
These properties were similar to the biochemical features of P301L mutated human tau in a mouse model of tauopathy.
|
29772786 |
2018 |
Tauopathies
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|
0.100 |
GeneticVariation
|
BEFREE |
Here, we showed that Aβ-induced tau hyperphosphorylation and neurodegeneration, including tau phosphorylation, synaptic disorder and neuronal loss, in the brains of both male wild-type (Wt) mice and male P301L transgenic mice (a mouse model of human tauopathy) were alleviated by genetic knockout of p75<sup>NTR</sup> in the both mouse models.
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31394202 |
2019 |
Tauopathies
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|
0.100 |
GeneticVariation
|
BEFREE |
Overall, our genetically matched mice have revealed that 4R NM hTau over expression is pathogenic in a manner distinct from classical aging-related tauopathy, underlining the importance of assaying the effects of transgenic disease-related proteins at appropriate stages in life.<b>SIGNIFICANCE STATEMENT</b>Due to differences in creation of transgenic lines, the pathological properties the P301L mutation confers to the tau protein <i>in vivo</i> have remained elusive, perhaps contributing to the lack of disease-modifying therapies for tauopathies.
|
31685653 |
2020 |