Frontotemporal dementia
|
0.700 |
Biomarker
|
disease |
BEFREE |
Frontotemporal dementia-associated protein "phosphorylated TDP-43" localizes to atherosclerotic lesions of human carotid and main cerebral arteries.
|
31259382 |
2020 |
Frontotemporal dementia
|
0.700 |
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 |
Frontotemporal dementia
|
0.700 |
Biomarker
|
disease |
BEFREE |
Genetic and immunopathological analysis of CHCHD10 in Australian amyotrophic lateral sclerosis and frontotemporal dementia and transgenic TDP-43 mice.
|
31690696 |
2020 |
Frontotemporal dementia
|
0.700 |
Biomarker
|
disease |
BEFREE |
Together with our observation that ATG7 is reduced in ALS-FTD brain tissues, these findings identify the autophagy pathway as one key effector of nuclear depletion of TARDBP that contributes to neurodegeneration.
|
31242080 |
2020 |
Frontotemporal dementia
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
Indeed, a growing number of FTD and/or ALS related RBPs coding genes (TDP43, FUS, EWSR1, TAF15, hnRNPA1, hnRNPA2B1, ATXN2, TIA1) have been identified to interfere with SG formation through mutation of their low-complexity domain (LCD), and thereby cause or influence disease.
|
31626953 |
2020 |
Frontotemporal dementia
|
0.700 |
Biomarker
|
disease |
BEFREE |
Frontotemporal lobar degeneration with TDP-43 immunoreactive (TDP-ir) inclusions (FTLD-TDP) is sub-classified based on the pattern of neocortical pathology, with each subtype showing clinical and genetic correlations.
|
31501924 |
2020 |
Frontotemporal dementia
|
0.700 |
Biomarker
|
disease |
BEFREE |
Prominent examples are amyloid beta and tau in Alzheimer's disease, α-synuclein in Parkinson's disease and transactive response DNA binding protein 43 kDa (TDP-43) in ALS and FTD.
|
31132469 |
2020 |
Frontotemporal dementia
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
Here we provide a statistically rigorous method to account for staining batch effects in a large sample of brain tissue with frontotemporal lobar degeneration with tau inclusions (FTLD-Tau, <i>N</i> = 39) or TDP-43 inclusions (FTLD-TDP, <i>N</i> = 53).
|
31333403 |
2019 |
Frontotemporal dementia
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
Different morphological characteristics of TDP-43 immunoreactive inclusions define the different variants of FTLD-TDP.
|
31266542 |
2019 |
Frontotemporal dementia
|
0.700 |
Biomarker
|
disease |
BEFREE |
Thus, premature polyadenylation-mediated reduction in stathmin-2 is a hallmark of ALS-FTD that functionally links reduced nuclear TDP-43 function to enhanced neuronal vulnerability.
|
30643298 |
2019 |
Frontotemporal dementia
|
0.700 |
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 |
Frontotemporal dementia
|
0.700 |
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 |
Frontotemporal dementia
|
0.700 |
Biomarker
|
disease |
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 |
Frontotemporal dementia
|
0.700 |
Biomarker
|
disease |
BEFREE |
We developed transgenic mice conditionally overexpressing human wild-type TDP-43 protein (hTDP-43-WT) in forebrain neurons, a model that recapitulates several key features of FTD.
|
31068973 |
2019 |
Frontotemporal dementia
|
0.700 |
Biomarker
|
disease |
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 |
Frontotemporal dementia
|
0.700 |
Biomarker
|
disease |
BEFREE |
Defects in nucleocytoplasmic transport in FTD point to important commonalities in the pathogenic mechanisms of tau-mediated dementias and ALS-FTD due to TDP-43 and C9orf72 mutations.
|
30650353 |
2019 |
Frontotemporal dementia
|
0.700 |
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 |
Frontotemporal dementia
|
0.700 |
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 |
Frontotemporal dementia
|
0.700 |
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 |
Frontotemporal dementia
|
0.700 |
Biomarker
|
disease |
BEFREE |
Recently, we further showed that these specialized neurons show preferential aggregation of TDP-43 in FTLD-TDP.
|
31640778 |
2019 |
Frontotemporal dementia
|
0.700 |
Biomarker
|
disease |
BEFREE |
These studies demonstrate the significance of the salt bridge in sustaining TDP43 stability and RNA binding properties, factors that are crucial for neurodegeneration arising from TDP43 deposition in ALS and FTD.
|
31018129 |
2019 |
Frontotemporal dementia
|
0.700 |
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 |
Frontotemporal dementia
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
In amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), pathology is often associated with aggregation of TAR DNA-binding protein 43 (TDP-43).
|
31495585 |
2019 |
Frontotemporal dementia
|
0.700 |
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 |
Frontotemporal dementia
|
0.700 |
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 |