Neurodegenerative Disorders
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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 |
Neurodegenerative Disorders
|
0.100 |
Biomarker
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group |
BEFREE |
TDP-43 aggregates are a salient feature for ALS, FTD and a variety of other neurodegenerative diseases including AD.
|
31846303 |
2020 |
Neurodegenerative Disorders
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0.100 |
Biomarker
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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.
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31376286 |
2020 |
Neurodegenerative Disorders
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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 |
Neurodegenerative Disorders
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0.100 |
Biomarker
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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 |
Neurodegenerative Disorders
|
0.100 |
GeneticVariation
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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 |
Neurodegenerative Disorders
|
0.100 |
Biomarker
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group |
BEFREE |
Stress-induced misfolding and intraneuronal aggregation of the highly conserved nucleic acid binding protein TDP-43 (transactive response DNA binding protein 43 kDa) and its fragments have been implicated in amyotrophic lateral sclerosis and several other neurodegenerative diseases.
|
30520297 |
2019 |
Neurodegenerative Disorders
|
0.100 |
Biomarker
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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 |
Neurodegenerative Disorders
|
0.100 |
Biomarker
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group |
BEFREE |
Aberrant function of the RNA-binding protein TDP-43 has been causally linked to multiple neurodegenerative diseases.
|
30692134 |
2019 |
Neurodegenerative Disorders
|
0.100 |
Biomarker
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group |
BEFREE |
Insoluble aggregates containing TDP-43 are widely observed in the diseased brain, and defined as "TDP-43 pathology" in a spectrum of neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS), Alzheimer's disease and ALS with frontotemporal dementia.
|
30450515 |
2019 |
Neurodegenerative Disorders
|
0.100 |
Biomarker
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group |
BEFREE |
Pathological TDP-43 aggregation has been found in ∼98% ALS and other neurodegenerative diseases including Alzheimer's.
|
31079926 |
2019 |
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 |
Neurodegenerative Disorders
|
0.100 |
Biomarker
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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 |
Neurodegenerative Disorders
|
0.100 |
AlteredExpression
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group |
BEFREE |
These results may shed light on the link between dysregulation of TDP-43-mediated mRNA deadenylation and pathogenesis of neurodegenerative diseases.
|
30520513 |
2019 |
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 |
Neurodegenerative Disorders
|
0.100 |
Biomarker
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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 |
Neurodegenerative Disorders
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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 |
Neurodegenerative Disorders
|
0.100 |
Biomarker
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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 |
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 |
Neurodegenerative Disorders
|
0.100 |
Biomarker
|
group |
BEFREE |
The process of misfolding and aggregation of neuronal proteins such as α-synuclein, Tau, amyloid beta (Aβ), TDP-43 or SOD1 is a common hallmark of many neurodegenerative disorders and iron has been shown to facilitate protein aggregation.
|
30723395 |
2019 |
Neurodegenerative Disorders
|
0.100 |
Biomarker
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group |
BEFREE |
Here, we review advances establishing that NIRs also function in the cytoplasm to prevent and reverse functional and aberrant phase transitions of their cargo, including neurodegenerative disease-linked RNA-binding proteins (RBPs) with prion-like domains, such as TDP-43, FUS, hnRNPA1, and hnRNPA2.
|
30660504 |
2019 |
Neurodegenerative Disorders
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0.100 |
Biomarker
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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 |
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 |
Neurodegenerative Disorders
|
0.100 |
Biomarker
|
group |
BEFREE |
TAR DNA-binding protein 43 (TDP-43) inclusions have been found in Amyotrophic lateral sclerosis (ALS) and several other neurodegenerative diseases.
|
29279008 |
2019 |
Neurodegenerative Disorders
|
0.100 |
Biomarker
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group |
BEFREE |
TDP-43 (transactive- response DNA binding protein) amazes structural biologist as its aberrant ubiquitinated cytosolic inclusions is largely involved in neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD).
|
30315897 |
2019 |