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Pick Disease of the Brain
|
0.100 |
Biomarker
|
disease |
BEFREE |
Genetic and immunopathological analysis of CHCHD10 in Australian amyotrophic lateral sclerosis and frontotemporal dementia and transgenic TDP-43 mice.
|
31690696 |
2020 |
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Pick Disease of the Brain
|
0.100 |
Biomarker
|
disease |
BEFREE |
Frontotemporal dementia-associated protein "phosphorylated TDP-43" localizes to atherosclerotic lesions of human carotid and main cerebral arteries.
|
31259382 |
2020 |
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Pick Disease of the Brain
|
0.100 |
Biomarker
|
disease |
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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Pick Disease of the Brain
|
0.100 |
Biomarker
|
disease |
BEFREE |
The TAR DNA-binding protein (TDP-43) self-assembles into prion-like aggregates considered to be the structural hallmark of amyotrophic lateral sclerosis and frontotemporal dementia.
|
31782904 |
2019 |
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Pick Disease of the Brain
|
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 |
|
Pick Disease of the Brain
|
0.100 |
Biomarker
|
disease |
BEFREE |
While cytoplasmic aggregation of TDP-43 is a pathological hallmark of amyotrophic lateral sclerosis and frontotemporal dementia, how aggregates form and what drives its nuclear clearance have not been determined.
|
30853299 |
2019 |
|
Pick Disease of the Brain
|
0.100 |
Biomarker
|
disease |
BEFREE |
Pathological aggregation of the transactive response DNA-binding protein of 43 kDa (TDP-43) is associated with several neurodegenerative disorders, including ALS, frontotemporal dementia, chronic traumatic encephalopathy, and Alzheimer's disease.
|
30814253 |
2019 |
|
Pick Disease of the Brain
|
0.100 |
Biomarker
|
disease |
BEFREE |
Accumulation of TDP-43 in the cytoplasm of diseased neurons is the pathological hallmark of frontotemporal dementia-TDP (FTLD-TDP) and amyotrophic lateral sclerosis (ALS), two diseases that lack efficacious medicine to prevent or to stop disease progression.
|
30785719 |
2019 |
|
Pick Disease of the Brain
|
0.100 |
Biomarker
|
disease |
BEFREE |
The RNA-binding protein TDP-43, associated to amyotrophic lateral sclerosis and frontotemporal dementia, regulates the alternative splicing of several genes, including the skipping of TNIK exon 15.
|
31382054 |
2019 |
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Pick Disease of the Brain
|
0.100 |
Biomarker
|
disease |
BEFREE |
Here we constructed a conditional TDP-43 mouse with depletion of TDP-43 in the mouse forebrain and find that the mice exhibit a whole spectrum of age-dependent frontotemporal dementia-like behaviour abnormalities including perturbation of social behaviour, development of dementia-like behaviour, changes of activities of daily living, and memory loss at a later stage of life.
|
30922385 |
2019 |
|
Pick Disease of the Brain
|
0.100 |
Biomarker
|
disease |
BEFREE |
<sup>18</sup>F-Flortaucipir in TDP-43 associated frontotemporal dementia.
|
30988363 |
2019 |
|
Pick Disease of the Brain
|
0.100 |
Biomarker
|
disease |
BEFREE |
Cytoplasmic aggregates and nuclear depletion of the ubiquitous RNA-binding protein TDP-43 have been described in the autoptic brain tissues of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTLD) patients and both TDP-43 loss-of-function and gain-of-function mechanisms seem to contribute to the neurodegenerative process.
|
31766750 |
2019 |
|
Pick Disease of the Brain
|
0.100 |
Biomarker
|
disease |
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 |
|
Pick Disease of the Brain
|
0.100 |
Biomarker
|
disease |
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 |
|
Pick Disease of the Brain
|
0.100 |
Biomarker
|
disease |
BEFREE |
There were no significant differences between clinical syndromes (PPA subtypes), the main clinical forms of dementia (frontotemporal dementia and AD), or the expected pathological groups (frontotemporal lobar degeneration-tau [FTLD-tau], FTLD-TDP43, and AD).
|
31640103 |
2019 |
|
Pick Disease of the Brain
|
0.100 |
Biomarker
|
disease |
BEFREE |
Frontotemporal dementia is a group of early onset dementia syndromes linked to underlying frontotemporal lobar degeneration (FTLD) pathology that can be classified based on the formation of abnormal protein aggregates involving tau and two RNA binding proteins, TDP-43 and FUS.
|
30355151 |
2019 |
|
Pick Disease of the Brain
|
0.100 |
Biomarker
|
disease |
BEFREE |
Numerous patient mutations in TARDBP, the gene encoding TDP-43, combined with data from animal and cell-based models, imply that altered RNA regulation by TDP-43 causes Amyotrophic Lateral Sclerosis and Frontotemporal Dementia.
|
30357366 |
2019 |
|
Pick Disease of the Brain
|
0.100 |
Biomarker
|
disease |
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 |
|
Pick Disease of the Brain
|
0.100 |
Biomarker
|
disease |
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 |
|
Pick Disease of the Brain
|
0.100 |
Biomarker
|
disease |
BEFREE |
TDP43 nuclear export and neurodegeneration in models of amyotrophic lateral sclerosis and frontotemporal dementia.
|
29545601 |
2018 |
|
Pick Disease of the Brain
|
0.100 |
Biomarker
|
disease |
BEFREE |
TAR DNA-binding protein 43 (TDP-43) is a major protein component of pathological inclusions in amyotrophic lateral sclerosis and frontotemporal dementia.
|
30372676 |
2018 |
|
Pick Disease of the Brain
|
0.100 |
Biomarker
|
disease |
BEFREE |
TDP-43 inclusions also characterize patients with GGGGCC (G4C2) hexanucleotide repeat expansion in C9orf72 that causes the most common genetic form of amyotrophic lateral sclerosis and frontotemporal dementia (C9ALS/FTD).
|
30239641 |
2018 |
|
Pick Disease of the Brain
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Whereas wild-type UBQLN2 accumulates in intraneuronal deposits in several common age-related neurodegenerative diseases, mutations in the gene encoding this protein result in X-linked amyotrophic lateral sclerosis/frontotemporal dementia associated with TDP43 accumulation.
|
30333186 |
2018 |
|
Pick Disease of the Brain
|
0.100 |
Biomarker
|
disease |
BEFREE |
Oligomerisation of Aβ, tau, α-synuclein and TDP-43 leads to a toxin gain- or loss-of-function contributing to the phenotype observed in Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis and frontotemporal dementia.
|
29986938 |
2018 |
|
Pick Disease of the Brain
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Fragments of the TAR DNA-binding protein 43 (TDP43) are major components of intracellular aggregates associated with amyotrophic lateral sclerosis and frontotemporal dementia.
|
29987190 |
2018 |