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TDP-43 Proteinopathies
|
0.100 |
Biomarker
|
disease |
BEFREE |
In AD patients and 5XFAD mice, the level of TDP-43 is increased in mitochondria, and TDP-43 highly co-localizes with mitochondria in brain neurons exhibiting TDP-43 proteinopathy.
|
31678156 |
2020 |
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TDP-43 Proteinopathies
|
0.100 |
Biomarker
|
disease |
BEFREE |
Our results show that TDP-43-WT mice have a phenotype that qualifies them as a useful model of FTD and provide valuable information for susceptibility windows in therapeutic strategies for TDP-43 proteinopathies.
|
31068973 |
2019 |
|
TDP-43 Proteinopathies
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Neuropathological evaluation of both cases revealed frontotemporal lobar degeneration with TDP-43 proteinopathy type B and selective involvement of upper motor neurons with TDP-43 inclusions.
|
31244341 |
2019 |
|
TDP-43 Proteinopathies
|
0.100 |
Biomarker
|
disease |
BEFREE |
PTK2/FAK regulates UPS impairment via SQSTM1/p62 phosphorylation in TARDBP/TDP-43 proteinopathies.
|
31690171 |
2019 |
|
TDP-43 Proteinopathies
|
0.100 |
Biomarker
|
disease |
BEFREE |
C9orf72-mutation carriers exhibited none or limited <sup>18</sup>F-Flortaucipir retention, indicating that <sup>18</sup>F-Flortaucipir binding in TDP-43 proteinopathies is not a general TDP-43 related phenomenon.
|
30988363 |
2019 |
|
TDP-43 Proteinopathies
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
RRM adjacent TARDBP mutations disrupt RNA binding and enhance TDP-43 proteinopathy.
|
31605140 |
2019 |
|
TDP-43 Proteinopathies
|
0.100 |
Biomarker
|
disease |
BEFREE |
Together, our results demonstrate that TDP-43 induced mitochondrial impairment is a critical aspect in TDP-43 proteinopathy.
|
31100073 |
2019 |
|
TDP-43 Proteinopathies
|
0.100 |
Biomarker
|
disease |
BEFREE |
Therapeutic approaches that inhibit SG formation may therefore be effective at suppressing TDP-43-mediated toxicity in patients with ALS and related TDP-43 proteinopathies.
|
30630951 |
2019 |
|
TDP-43 Proteinopathies
|
0.100 |
Biomarker
|
disease |
BEFREE |
Here, we review the current status of research pursued to identify specific indicators to predict or exclude TDP-43 pathology in the ALS-FTLD spectrum disorders and findings on candidates for prognosis and monitoring of disease progression in TDP-43 proteinopathies with a focus on TDP-43 with its pathological forms, neurochemical and imaging biomarkers.
|
30399416 |
2019 |
|
TDP-43 Proteinopathies
|
0.100 |
Biomarker
|
disease |
BEFREE |
The decrease in the efficiency of the clearance systems in aging, as well as the presence of genetic mutations of proteins associated with cellular proteostasis in the familial forms of TDP-43 proteinopathies, suggest that a failure of these protein degradation systems is a key factor in the aetiology of TDP-43 associated disorders.
|
31357627 |
2019 |
|
TDP-43 Proteinopathies
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
To identify genetic modifiers of TDP-43 neurotoxicity, we utilized a Caenorhabditis elegans model of TDP-43 proteinopathy expressing human mutant TDP-43 pan-neuronally (TDP-43 tg).
|
31834878 |
2019 |
|
TDP-43 Proteinopathies
|
0.100 |
Biomarker
|
disease |
BEFREE |
TDP-43 proteinopathy is a pathological hallmark of amyotrophic lateral sclerosis and frontotemporal dementia where cytoplasmic TDP-43 inclusions are observed within degenerating regions of patient postmortem tissue.
|
30826182 |
2019 |
|
TDP-43 Proteinopathies
|
0.100 |
Biomarker
|
disease |
BEFREE |
Although the pathomechanisms underlying TDP-43 proteinopathy remain elusive, pathologically relevant TDP-43 has been repeatedly shown to be present in either the inside or outside of mitochondria, and functionally involved in the regulation of mitochondrial morphology, trafficking, and function, suggesting mitochondria as likely targets of TDP-43 proteinopathy.
|
31445085 |
2019 |
|
TDP-43 Proteinopathies
|
0.100 |
Biomarker
|
disease |
BEFREE |
Mislocalization of TDP-43 from the nucleus to the cytoplasm is an early step of TDP-43 proteinopathy.
|
29615863 |
2018 |
|
TDP-43 Proteinopathies
|
0.100 |
Biomarker
|
disease |
BEFREE |
Our findings indicate that components of the carnitine shuttle are misexpressed in the context of TDP-43 proteinopathy and that genetic modulation of CPT1 or CPT2 expression, two core components of the carnitine shuttle, mitigates TDP-43 dependent locomotor dysfunction, in a variant dependent manner.
|
29904341 |
2018 |
|
TDP-43 Proteinopathies
|
0.100 |
Biomarker
|
disease |
BEFREE |
We interrogated pathological Alzheimer's disease (n = 247); other tauopathies (n = 95) including Pick's disease, corticobasal disease and progressive supranuclear palsy; the synucleinopathies (n = 164) including multiple system atrophy and Lewy body disease; the TDP-43 proteinopathies (n = 188) including frontotemporal lobar degeneration with TDP-43 inclusions and amyotrophic lateral sclerosis; and a minimal pathology group (n = 72).
|
29878075 |
2018 |
|
TDP-43 Proteinopathies
|
0.100 |
Biomarker
|
disease |
BEFREE |
The acetylated inclusions containing TDP-43 are found in the spinal cord of amyotrophic lateral sclerosis (ALS) patients, suggesting that aberrant TDP-43 acetylation and resulting disruption of RNA binding are linked to onset and progression of TDP-43 proteinopathy.
|
29866003 |
2018 |
|
TDP-43 Proteinopathies
|
0.100 |
Biomarker
|
disease |
BEFREE |
Surprisingly, the TTBK2/TDP-43 transgenic combination showed no exacerbation of TDP-43 proteinopathy related phenotypes.
|
29409526 |
2018 |
|
TDP-43 Proteinopathies
|
0.100 |
Biomarker
|
disease |
BEFREE |
Here, after a discussion of stages of TDP-43 proteinopathy during disease progression in various major neurodegenerative diseases, we review previous and most recent studies about the potential pathomechanisms with a particular emphasis on ALS, FTLD, and AD, and discuss the possibility of targeting TDP-43 as a common therapeutic approach to treat neurodegenerative diseases.
|
29486049 |
2018 |
|
TDP-43 Proteinopathies
|
0.100 |
Biomarker
|
disease |
BEFREE |
Our findings highlight that the phosphatase regulator, GADD34, also functions as a kinase scaffold in response to chronic oxidative stress and recruits CK1ϵ and oxidized TDP-43 to facilitate its phosphorylation, as seen in TDP-43 proteinopathies.
|
29109149 |
2018 |
|
TDP-43 Proteinopathies
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Selected compounds were initially screened for anti-convulsant activity in a C. elegans pentylenetetrazol-induced seizure assay, as a rapid primary readout of bioactivity; and then assessed for neuroprotective properties in a C. elegans TDP-43 proteinopathy model based on pan-neuronal expression of human A315T mutant TDP-43.
|
29940336 |
2018 |
|
TDP-43 Proteinopathies
|
0.100 |
Biomarker
|
disease |
BEFREE |
Although aggregation-prone TAR DNA-binding protein 43 (TDP-43) has been recognized as a major component of the ubiquitin pathology, the mechanisms by which UBQLN involves in TDP-43 proteinopathy have not yet been elucidated in detail.
|
29936333 |
2018 |
|
TDP-43 Proteinopathies
|
0.100 |
Biomarker
|
disease |
BEFREE |
APOE ε4 seems to increase TDP-43 burden, and this effect in turn was associated with higher odds of hippocampal sclerosis, a pathology potentially downstream of TDP-43 proteinopathy.
|
30093249 |
2018 |
|
TDP-43 Proteinopathies
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
The present review focuses first on general aspects of oligodendrocytes and precursors, and their development and functions, and then introduces and updates alterations and dysfunction of oligodendrocytes in selected neurodegenerative diseases with abnormal protein aggregates such as multiple system atrophy, Lewy body diseases, tauopathies, Alzheimer's disease, amyotrophic lateral sclerosis, frontotemporal lobar degeneration with TDP-43 inclusions (TDP-43 proteinopathies), and Creutzfeldt-Jakob´s disease as a prototypical human prionopathy.
|
30077775 |
2018 |
|
TDP-43 Proteinopathies
|
0.100 |
Biomarker
|
disease |
BEFREE |
There are no effective TDP-43-directed therapies for ALS or related TDP-43 proteinopathies, such as frontotemporal dementia.
|
28405022 |
2017 |