|
Frontotemporal Lobar Degeneration
|
0.400 |
Biomarker
|
disease |
BEFREE |
Distinct TDP-43 inclusion morphologies in the anterior cingulate cortex of FTLD and FTLD-ALS may be linked to heterogeneity in the ubiquitination of pathological TDP-43 inclusions, with the present study providing evidence to suggest the involvement of distinct pathomechanisms in these two overlapping clinical syndromes.
|
29078806 |
2017 |
|
Frontotemporal Lobar Degeneration
|
0.400 |
Biomarker
|
disease |
BEFREE |
Here, we review the current status of research pursued to identify specific indicators to predict or exclude TDP-43 pathology in the ALS-FTLD spectrum disorders and findings on candidates for prognosis and monitoring of disease progression in TDP-43 proteinopathies with a focus on TDP-43 with its pathological forms, neurochemical and imaging biomarkers.
|
30399416 |
2019 |
|
Frontotemporal Lobar Degeneration
|
0.400 |
Biomarker
|
disease |
BEFREE |
The identification of pathologic TDP-43 aggregates in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration, followed by the discovery of dominantly inherited point mutations in TDP-43 in familial ALS, have been critical insights into the mechanism of these untreatable neurodegenerative diseases.
|
20554523 |
2010 |
|
Frontotemporal Lobar Degeneration
|
0.400 |
Biomarker
|
disease |
BEFREE |
In this article, we will review the contemporary clinical, genetic and neuropathological characteristics of the frontotemporal syndromes of ALS and propose that as opposed to being a FTLD in which TDP-43 is the primary disease protein (FTLD-TDP) and that the frontotemporal syndromes of ALS represent a hybrid of both TDP-43 and tau pathology.
|
21809041 |
2011 |
|
Frontotemporal Lobar Degeneration
|
0.400 |
Biomarker
|
disease |
BEFREE |
Understanding the basis of the proposed mechanisms and shared pathways, as well as interactions with known key proteins such as TAR DNA-binding protein (TDP-43) are needed to clarify the pathology of ALS and/or FTLD, and make possible steps toward therapy development.
|
24836899 |
2014 |
|
Frontotemporal Lobar Degeneration
|
0.400 |
Biomarker
|
disease |
BEFREE |
We determined whether there are distinct TDP-43 types in non-FTLD brains.
|
30604226 |
2019 |
|
Frontotemporal Lobar Degeneration
|
0.400 |
Biomarker
|
disease |
BEFREE |
To achieve this, we systematically screened ALS/FTLD-associated and synthetic TDP-43 isoforms via same-site gene insertion and neural expression in Drosophila; followed by transposon-based motor neuron-specific transgenesis in a chick vertebrate system.
|
20806063 |
2010 |
|
Frontotemporal Lobar Degeneration
|
0.400 |
Biomarker
|
disease |
BEFREE |
Tar DNA binding protein 43 (TDP-43) is the major component of pathological deposits in frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) and in amyotrophic lateral sclerosis (ALS).
|
23922830 |
2013 |
|
Frontotemporal Lobar Degeneration
|
0.400 |
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 |
|
Frontotemporal Lobar Degeneration
|
0.400 |
Biomarker
|
disease |
BEFREE |
Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are characterized by cytoplasmic protein aggregates in the brain and spinal cord that include TAR-DNA binding protein 43 (TDP-43).
|
21206091 |
2011 |
|
Frontotemporal Lobar Degeneration
|
0.400 |
Biomarker
|
disease |
BEFREE |
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
|
17023659 |
2006 |
|
Frontotemporal Lobar Degeneration
|
0.400 |
Biomarker
|
disease |
BEFREE |
Mutations in one of these genes (GRN), which encodes progranulin, have been implicated in up to a quarter of cases of frontotemporal lobar degeneration with TDP-43 (TAR DNA-binding protein 43)-positive inclusions; currently, there are more than 60 known pathogenic mutations of the gene.
|
24709683 |
2014 |
|
Frontotemporal Lobar Degeneration
|
0.400 |
AlteredExpression
|
disease |
BEFREE |
Mimicking aspects of frontotemporal lobar degeneration and Lou Gehrig's disease in rats via TDP-43 overexpression.
|
19223871 |
2009 |
|
Frontotemporal Lobar Degeneration
|
0.400 |
Biomarker
|
disease |
BEFREE |
Mutations in the TAR DNA-binding protein 43 gene (TARDBP or TDP-43) may occur in 3-4% of FALS cases, and less frequently, in FTLD.
|
20655970 |
2011 |
|
Frontotemporal Lobar Degeneration
|
0.400 |
Biomarker
|
disease |
BEFREE |
The semantic variant of primary progressive aphasia (svPPA) is typically associated with frontotemporal lobar degeneration (FTLD) with longTAR DNA-binding protein (TDP)-43-positive neuropil threads and dystrophic neurites (type C), and is only rarely due to a primary tauopathy or Alzheimer's disease.
|
28986472 |
2018 |
|
Frontotemporal Lobar Degeneration
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
The role of mutant TAR DNA-binding protein 43 in amyotrophic lateral sclerosis and frontotemporal lobar degeneration.
|
21787329 |
2011 |
|
Frontotemporal Lobar Degeneration
|
0.400 |
Biomarker
|
disease |
BEFREE |
In this study, we analysed the influence of PEI-coated magnetic NPs designed for biotechnological applications and industrial SiO<sub>2</sub>, TiO<sub>2</sub> N and TiO<sub>2</sub> P25 NPs on intracellular localization and solubility of fused in farcoma (FUS) and TAR-DNA binding protein 43 (TDP-43) that are important pathological hallmarks of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD).
|
28444573 |
2017 |
|
Frontotemporal Lobar Degeneration
|
0.400 |
Biomarker
|
disease |
BEFREE |
To determine the clinical, anatomical, genetic and pathological features of dual frontotemporal lobar degeneration (FTLD) pathology: FTLD-tau and FTLD-TDP-43 in a large clinicopathological cohort.
|
30324308 |
2018 |
|
Frontotemporal Lobar Degeneration
|
0.400 |
Biomarker
|
disease |
BEFREE |
Frontotemporal lobar degeneration with motor neuron disease (FTLD-MND) is characterized by neuronal cytoplasmic inclusions containing TDP-43.
|
22512241 |
2013 |
|
Frontotemporal Lobar Degeneration
|
0.400 |
Biomarker
|
disease |
BEFREE |
TAR DNA-binding protein 43 (TDP-43) inclusions are pathological hallmarks of patients with frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS).
|
26555887 |
2015 |
|
Frontotemporal Lobar Degeneration
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Laminar distribution of the pathological changes in sporadic frontotemporal lobar degeneration with transactive response (TAR) DNA-binding protein of 43 kDa (TDP-43) proteinopathy: a quantitative study using polynomial curve fitting.
|
22804696 |
2013 |
|
Frontotemporal Lobar Degeneration
|
0.400 |
Biomarker
|
disease |
BEFREE |
Importantly, aggregation of misfolded FUS or TDP-43 is also characteristic of several neurodegenerative disorders in addition to ALS, including frontotemporal lobar degeneration.
|
25792726 |
2015 |
|
Frontotemporal Lobar Degeneration
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Heterozygous loss-of-function mutations in the progranulin (GRN) gene and the resulting reduction of GRN levels is a common genetic cause for frontotemporal lobar degeneration (FTLD) with accumulation of TAR DNA-binding protein (TDP)-43.
|
24619111 |
2014 |
|
Frontotemporal Lobar Degeneration
|
0.400 |
Biomarker
|
disease |
BEFREE |
Moreover, <i>GRN</i> loss-of-function mutations are causative of a subset of FTLD cases showing TDP-43 pathological aggregates.
|
31766750 |
2019 |
|
Frontotemporal Lobar Degeneration
|
0.400 |
Biomarker
|
disease |
BEFREE |
TAR DNA-binding protein of 43 kDa (TDP-43) is a major component of the pathological inclusions of frontotemporal lobar degeneration with TDP-43 proteinopathy, also called FTLD with ubiquitin-positive, tau-negative inclusions (FTLD-U), and motor neuron disease (MND).
|
18974920 |
2008 |