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Neurodegenerative Disorders
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0.100 |
Biomarker
|
group |
BEFREE |
TDP-43 aggregates are a salient feature for ALS, FTD and a variety of other neurodegenerative diseases including AD.
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31846303 |
2020 |
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Neurodegenerative Disorders
|
0.100 |
Biomarker
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group |
BEFREE |
Insoluble, hyperubiquitylated TAR DNA binding protein of 43 kDa (TDP-43) in the central nervous system characterizes frontotemporal dementia and ALS in many individuals with these neurodegenerative diseases.
|
31780563 |
2020 |
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Neurodegenerative Disorders
|
0.100 |
GeneticVariation
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group |
BEFREE |
Therefore, targeting the PTK2-TBK1-SQSTM1 axis may represent a novel therapeutic intervention for neurodegenerative diseases with TARDBP proteinopathies.<b>Abbreviations</b>: ALP: macroautophagy/autophagy lysosomal pathway; ALS: amyotrophic lateral sclerosis; ATXN2: ataxin 2; BafA1: bafilomycin A<sub>1</sub>; cCASP3: cleaved caspase 3; CSNK2: casein kinase 2; FTLD: frontotemporal lobar degeneration; MAP1LC3/LC3: microtubule-associated protein 1 light chain 3; OPTN: optineurin; PTK2/FAK: PTK2 protein tyrosine kinase 2; SQSTM1/p62: sequestosome 1; TARDBP/TDP-43: TAR DNA binding protein; TBK1: TANK binding kinase 1; ULK1: unc-51 like autophagy activating kinase 1; UPS: ubiquitin-proteasome system.
|
31690171 |
2019 |
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Neurodegenerative Disorders
|
0.100 |
Biomarker
|
group |
BEFREE |
A common pathological hallmark of amyotrophic lateral sclerosis (ALS) and the related neurodegenerative disorder frontotemporal dementia, is the cellular mislocalization of transactive response DNA-binding protein 43 kDa (TDP-43).
|
31642482 |
2019 |
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Neurodegenerative Disorders
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0.100 |
Biomarker
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group |
BEFREE |
These insights contribute to a better understanding of native biological functions of TDP-43 and FUS and potential molecular pathways in neurodegenerative diseases.
|
31479821 |
2019 |
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Neurodegenerative Disorders
|
0.100 |
Biomarker
|
group |
BEFREE |
Importantly, TDP-43 proteinopathy, characterized by aberrant phosphorylation, ubiquitination, cleavage or nuclear depletion of TDP-43 in neurons and glial cells, is a common prominent pathological feature of various major neurodegenerative diseases including ALS, FTD, and Alzheimer's disease (AD).
|
31445085 |
2019 |
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Neurodegenerative Disorders
|
0.100 |
Biomarker
|
group |
BEFREE |
Aggregation of TAR DNA-binding protein of 43 kDa (TDP-43) is a salient feature of amyotrophic lateral sclerosis (ALS), a debilitating neurodegenerative disorder affecting over 200 000 people worldwide.
|
31430111 |
2019 |
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Neurodegenerative Disorders
|
0.100 |
Biomarker
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group |
BEFREE |
Our data suggest a complex regulation between the ubiquitous TDP-43 and the neuron-specific NOVA-1 splicing factors in the brain that may help better understand the pathobiology of both neurodegenerative diseases and schizophrenia.
|
31382054 |
2019 |
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Neurodegenerative Disorders
|
0.100 |
Biomarker
|
group |
BEFREE |
Limbic-predominant age-related TAR-DNA-binding protein-43 (TDP-43) encephalopathy with hippocampal sclerosis pathology (LATE-NC + HS) is a neurodegenerative disorder characterized by severe hippocampal CA1 neuron loss and TDP-43-pathology, leading to cognitive dysfunction and dementia.
|
31376286 |
2020 |
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Neurodegenerative Disorders
|
0.100 |
Biomarker
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group |
BEFREE |
The inhibition of TDP-43 proteostasis in the presence of selective inhibitors against the proteasome and macroautophagy systems revealed that these two systems are both severely involved in TDP-43 accumulation and have a strong influence on each other in neurodegenerative disorders associated with TDP-43.
|
31357627 |
2019 |
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Neurodegenerative Disorders
|
0.100 |
Biomarker
|
group |
BEFREE |
TAR DNA-binding protein 43 kDa (TDP-43), encoded by TARDBP, is an RNA-binding protein, the nuclear depletion of which is the histopathological hallmark of amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disorder affecting both upper and lower motor neurons.
|
31355778 |
2019 |
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Neurodegenerative Disorders
|
0.100 |
Biomarker
|
group |
BEFREE |
TDP-43, a DNA/RNA binding protein, controls the expression of thousands of genes and is associated with several neurodegenerative diseases including amyotrophic lateral sclerosis, Alzheimer's disease, Huntington's disease, and chronic traumatic encephalopathy.
|
31316455 |
2019 |
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Neurodegenerative Disorders
|
0.100 |
Biomarker
|
group |
BEFREE |
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by accumulation of fragmented insoluble TDP-43 and loss of TDP-43 from the nucleus.
|
31310801 |
2019 |
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Neurodegenerative Disorders
|
0.100 |
Biomarker
|
group |
BEFREE |
The involvement of transactivation response (TAR) DNA-binding protein 43 (TDP-43) in neurodegenerative diseases was revealed in 2006, when it was first reported to be the main component of the intracellular inclusions in patients with amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration.
|
31287959 |
2019 |
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Neurodegenerative Disorders
|
0.100 |
Biomarker
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group |
BEFREE |
Here we demonstrate that another hnRNP family member, hnRNP E2, shows a striking accumulation within dystrophic neurites and cytoplasmic inclusions in the frontal cortex and hippocampus of a subset of FTLD-TDP cases belonging to pathological subtypes A and C, where hnRNP E2 was found to co-localize with 87% of TDP-43 immunopositive inclusions. hnRNP E2-positive inclusions were not seen in FTLD-TDP cases with the <i>C9orf72</i> expansion or in any other neurodegenerative disorders examined.
|
31213972 |
2019 |
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Neurodegenerative Disorders
|
0.100 |
Biomarker
|
group |
BEFREE |
TAR DNA-binding protein 43 (TDP-43) is a hallmark of some neurodegenerative disorders, such as frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
|
31176717 |
2019 |
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Neurodegenerative Disorders
|
0.100 |
GeneticVariation
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group |
BEFREE |
Mutations in or dys-regulation of the TDP-43 gene have been associated with TDP-43 proteinopathy, a spectrum of neurodegenerative diseases including Frontotemporal Lobar Degeneration (FTLD) and Amyotrophic Lateral Sclerosis (ALS).
|
31100073 |
2019 |
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Neurodegenerative Disorders
|
0.100 |
Biomarker
|
group |
BEFREE |
Pathological TDP-43 aggregation has been found in ∼98% ALS and other neurodegenerative diseases including Alzheimer's.
|
31079926 |
2019 |
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Neurodegenerative Disorders
|
0.100 |
GeneticVariation
|
group |
BEFREE |
Dysregulation of TAR DNA-binding protein 43 (TDP-43) is a hallmark feature of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS), two fatal neurodegenerative diseases.
|
31068973 |
2019 |
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Neurodegenerative Disorders
|
0.100 |
Biomarker
|
group |
BEFREE |
The structural integrity of N-terminal domain (NTD) of TAR DNA-binding protein-43 (TDP-43) is essential for the biological functions of TDP-43 involved in neurodegenerative diseases.
|
31035038 |
2019 |
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Neurodegenerative Disorders
|
0.100 |
Biomarker
|
group |
BEFREE |
Here, we review the scope of tau and TDP-43 phosphorylation in neurodegenerative disease and discuss recent work demonstrating the kinases TTBK1 and TTBK2 phosphorylate both tau and TDP-43, promoting neurodegeneration.
|
31034749 |
2019 |
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Neurodegenerative Disorders
|
0.100 |
Biomarker
|
group |
BEFREE |
Phase-separated compartments can concentrate specific RNA-binding proteins (RBPs), such as TDP-43 and fused in sarcoma (FUS), that through low-complexity, prion-like domains have an intrinsic tendency to form self-templating fibrils that are closely tied to fatal neurodegenerative disorders such as amyotrophic lateral sclerosis (ALS) and frontotemporal dementia.
|
30948513 |
2019 |
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Neurodegenerative Disorders
|
0.100 |
GeneticVariation
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group |
BEFREE |
In addition, wild-type TDP-43 is also frequently found in neuronal cytoplasmic aggregates in patients with neurodegenerative diseases not caused by TDP-43 mutations.
|
30905713 |
2019 |
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Neurodegenerative Disorders
|
0.100 |
GeneticVariation
|
group |
BEFREE |
Aggregates of the RNA-binding protein TDP-43 (TAR DNA-binding protein) are a hallmark of the overlapping neurodegenerative disorders amyotrophic lateral sclerosis (ALS) and frontotemporal dementia.
|
30824544 |
2019 |
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Neurodegenerative Disorders
|
0.100 |
Biomarker
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group |
BEFREE |
These combined results strongly suggest that LLPS may play a major role in pathological TDP-43 aggregation, contributing to pathogenesis in neurodegenerative diseases.
|
30814253 |
2019 |