|
GRN-related frontotemporal dementia
|
0.100 |
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 |
|
GRN-related frontotemporal dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
Brain sections from 5 PPA participants with postmortem diagnoses of frontotemporal lobar degeneration with TDP-43 pathology (FTLD-TDP) were immunohistochemically stained using an antibody to phosphorylated TDP-43 and quantitatively examined for regional and hemispheric distribution using unbiased stereology.
|
30753613 |
2019 |
|
GRN-related frontotemporal dementia
|
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 |
|
GRN-related frontotemporal dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
Antemortem volume loss mirrors TDP-43 staging in older adults with non-frontotemporal lobar degeneration.
|
31562527 |
2019 |
|
GRN-related frontotemporal dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
TDP-43 extracted from frontotemporal lobar degeneration subject brains displays distinct aggregate assemblies and neurotoxic effects reflecting disease progression rates.
|
30559480 |
2019 |
|
GRN-related frontotemporal dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
In Alzheimer's disease, protein TDP-43 may co-aggregate, but it is not clear whether this is atypical isolated Alzheimer's disease or overlap of Alzheimer's disease with early frontotemporal lobar degeneration.
|
31400306 |
2019 |
|
GRN-related frontotemporal dementia
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
TAR DNA-binding protein 43 (TDP-43) is an RNA-binding protein, whose loss-of-function mutation causes amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration.
|
30520513 |
2019 |
|
GRN-related frontotemporal dementia
|
0.100 |
Biomarker
|
disease |
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 |
|
GRN-related frontotemporal dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
Suppression of Progranulin Expression Leads to Formation of Intranuclear TDP-43 Inclusions In Vitro: A Cell Model of Frontotemporal Lobar Degeneration.
|
31626287 |
2019 |
|
GRN-related frontotemporal dementia
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Phosphorylated transactivation response DNA-binding protein 43 kDa (p-TDP-43)-immunoreactive neuronal and glial cytoplasmic inclusions are a histopathological hallmark of sporadic amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with TDP-43.
|
31020724 |
2019 |
|
GRN-related frontotemporal dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
More than half of the patients with Alzheimer's disease (AD) have comorbidities including TDP-43 and Lewy bodies, which are also associated with frontotemporal lobar degeneration and dementia with Lewy bodies, respectively.
|
31127776 |
2019 |
|
GRN-related frontotemporal dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
FTD syndromes are characterized by lobar atrophy (frontotemporal lobar degeneration or FTLD) and the presence of either cellular TDP43 (FTLD-TDP), tau (FTLD-tau), or FUS aggregates, while extracellular β-amyloid plaques and hyperphosphorylated tau tangles develop in AD.
|
30467822 |
2019 |
|
GRN-related frontotemporal dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
We assessed the prevalence and distribution of GVD in cases with TDP-43-related frontotemporal lobar degeneration (FTLD-TDP) and amyotrophic lateral sclerosis (ALS-TDP).
|
31144027 |
2019 |
|
GRN-related frontotemporal dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
As TDP43 protein is one of the proteins pathologically involved in frontotemporal lobar degeneration, many studies have been performed to assess TDP43 protein diagnostic performances.
|
30374726 |
2019 |
|
GRN-related frontotemporal dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
Heterogeneous Nuclear Ribonucleoprotein E2 (hnRNP E2) Is a Component of TDP-43 Aggregates Specifically in the A and C Pathological Subtypes of Frontotemporal Lobar Degeneration.
|
31213972 |
2019 |
|
GRN-related frontotemporal dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
The Tar DNA-Binding Protein 43 (TDP-43) and its phosphorylated isoform (pTDP-43) are the major components associated with ubiquitin positive/Tau-negative inclusions found in neurons and glial cells of patients suffering of amyotrophic lateral sclerosis (ALS) or frontotemporal lobar degeneration-TDP-43 (FTLD-TDP).
|
30863908 |
2019 |
|
GRN-related frontotemporal dementia
|
0.100 |
Biomarker
|
disease |
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 |
|
GRN-related frontotemporal dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
Transactive response DNA-binding protein 43 kDa (TDP-43) was identified as a major disease-associated component in the brain of patients with amyotrophic lateral sclerosis (ALS), as well as the largest subset of patients with frontotemporal lobar degeneration with ubiquitinated inclusions (FTLD-U), which characteristically exhibits cytoplasmic inclusions that are positive for ubiquitin but negative for tau and α-synuclein.
|
31555895 |
2019 |
|
GRN-related frontotemporal dementia
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
In addition, we carried out targeted sequencing of a cohort of 37 patients with frontotemporal lobar degeneration with Transactive response DNA-binding protein 43 (TDP-43) subtype from the Netherlands Brain bank.
|
30103325 |
2018 |
|
GRN-related frontotemporal dementia
|
0.100 |
GeneticVariation
|
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 |
|
GRN-related frontotemporal dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
TDP-43 is the major disease protein associated with amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with ubiquitinated inclusions (FTLD-TDP).
|
30353006 |
2018 |
|
GRN-related frontotemporal dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
To quantitatively determine the density and distribution of activated microglia across cortical regions and hemispheres in the brains of primary progressive aphasia (PPA) participants with pathological diagnoses of frontotemporal lobar degeneration with transactive response DNA-binding protein-43 (TDP-43) inclusions and to examine the relationships between microglial densities, patterns of focal atrophy, (TDP-43) inclusions, and clinical phenotype.
|
29665116 |
2018 |
|
GRN-related frontotemporal dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
Transactive response (TAR) DNA-binding protein 43 (TDP-43) has emerged as an important contributor to amyotrophic lateral sclerosis and frontotemporal lobar degeneration.
|
29742622 |
2018 |
|
GRN-related frontotemporal dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
TAR DNA-Binding Protein 43 and Disrupted in Schizophrenia 1 Coaggregation Disrupts Dendritic Local Translation and Mental Function in Frontotemporal Lobar Degeneration.
|
29752072 |
2018 |
|
GRN-related frontotemporal dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
Transactive response DNA-binding protein 43kD (TDP-43) is a major component of tau-negative and ubiquitin-positive inclusions that characterize ALS (amyotrophic lateral sclerosis) and FTLD (frontotemporal lobar degeneration).
|
29421661 |
2018 |