Presenile dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
The pooled odds ratio (OR) for TBI on development of dementia, FTD and TDP-43 associated disease were 1.93 (95% CI 1.47-2.55, p < 0.001), 4.44 (95% CI 3.86-5.10, p < 0.001), and 2.97 (95% CI 1.35-6.53, p < 0.001).
|
30396335 |
2018 |
Presenile dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
The GRN-related form of frontotemporal lobar dementia is a proteinopathy characterized by the appearance of neuronal inclusions containing ubiquitinated and fragmented TDP-43 (encoded by TARDBP).
|
29053785 |
2017 |
Presenile dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
The case is unusual and instructive because of the co-existence of frequent cortical and diencephalic amyloid plaques with extensive TDP-43-positive histopathology in the setting of early-onset dementia and because it demonstrates that a positive cortical amyloid imaging signal in a subject with dementia does not necessarily establish that Alzheimer's disease is the sole cause.
|
24927705 |
2014 |
Presenile dementia
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Pathogenic TDP-43 gene (TARDBP) mutations have been identified in familial ALS kindreds, and here we report a TARDBP variant (A90V) in a FTLD/ALS patient with a family history of dementia.
|
18505686 |
2008 |
Presenile dementia
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
The DNA/RNA binding proteins TAR DNA-binding protein 43 (TDP-43) and fused-in-sarcoma (FUS) are genetically linked to amyotrophic lateral sclerosis and frontotemporal lobar dementia, while the inappropriate cytoplasmic accumulations of TDP-43 and FUS are observed in a growing number of late-onset pathologies including spinocerebellar ataxia 3, Alzheimer's and Huntington's diseases (HD).
|
23172908 |
2013 |
Presenile dementia
|
0.100 |
Biomarker
|
disease |
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.
|
31376286 |
2020 |
Presenile dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
Full-length TDP-43 forms toxic amyloid oligomers that are present in frontotemporal lobar dementia-TDP patients.
|
25215604 |
2014 |
Presenile dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
Perturbation of these remarkable liquid phases can lead to aggregates, such as those formed by the proteins TDP-43 and FUS, which are linked to ALS and other dementia.
|
28176659 |
2017 |
Presenile dementia
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Ubiquitin and TDP-43 immunohistochemistry was performed on postmortem tissue from sporadic ALS (n = 59), ALS with SOD1 mutations (n = 15), SOD-1-negative FALS (n = 11), and ALS with dementia (n = 26).
|
17469116 |
2007 |
Presenile dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
Over the past decade, the transactive response DNA-binding protein of 43 kDa (TDP-43) has been recognized as a major protein in normal and pathological ageing, increasing the risk of cognitive impairment and dementia.
|
31562527 |
2019 |
Presenile dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
Pathological neuronal inclusions of the 43-kDa TAR DNA-binding protein (TDP-43) are implicated in dementia and motor neuron disorders; however, the molecular mechanisms of the underlying cell loss remain poorly understood.
|
21471218 |
2011 |
Presenile dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
The DNA/RNA-binding proteins TDP-43 and FUS are found in protein aggregates in a growing number of neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS) and related dementia, but little is known about the neurotoxic mechanisms.
|
22848727 |
2012 |
Presenile dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
Therefore the C9orf72 hexanucleotide repeat expansion appears to be specific to TDP-43 driven amyotrophic lateral sclerosis and dementia.
|
23084342 |
2013 |
Presenile dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
Critically, TDP-43 autoregulation is perturbed, leading to a gain of TDP-43 function and altered splicing of Mapt, another pivotal dementia-associated gene.
|
29556029 |
2018 |
Presenile dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
However, it has been reported more recently that TDP-43 positive inclusions occur in other neurodegenerative disorders such as Alzheimer's disease, Dementia with Lewy Bodies and Parkinsonism dementia complex of Guam.
|
19283396 |
2009 |
Presenile dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
Factors associated with survival were studied in 84 neuropathologically documented cases of the pre-senile dementia frontotemporal dementia lobar degeneration (FTLD) with transactive response (TAR) DNA-binding protein of 43 kDa (TDP-43) proteinopathy (FTLD-TDP).
|
27543771 |
2016 |
Presenile dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
The intrinsically disordered C-terminal domain of TAR DNA-binding protein 43 (TDP-43), a protein involved in motor neuron disease and dementia lacks a dominant LLPS motif, however, and how this domain forms condensates is unclear.
|
29511089 |
2018 |
Presenile dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
The importance of the pathways varied, with the vascular pathway accounting for 32% of the association between age and dementia, wheraes the remaining three inter-related degenerative pathways together accounted for 68% (amyloid/tau, 24%; the Lewy body, 1%; and TDP-43/hippocampal sclerosis, 43%).
|
29944741 |
2018 |
Presenile dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
The multiple roles played by RNA binding proteins in neurodegeneration have become apparent following the discovery of TAR DNA binding protein 43 kDa (TDP-43) and fused in sarcoma/translocated in liposarcoma (FUS/TLS) involvement in amyotrophic lateral sclerosis and frontotemporal lobar dementia.
|
27015757 |
2016 |
Presenile dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
Dementia (p < 0.001) and TDP-43 immunopositivity of the granular cell layer of the dentate fascia (p < 0.001) were strongly associated with HS.
|
29614661 |
2018 |
Presenile dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
This experiment investigated morphological subtypes of pre-inclusions and their relationship with TDP-43 inclusions in primary progressive aphasia (PPA), a dementia characterized by gradual dissolution of language.
|
30753613 |
2019 |
Presenile dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
TDP-43 and Alzheimer's Disease Pathologic Subtype in Non-Amnestic Alzheimer's Disease Dementia.
|
30010126 |
2018 |
Presenile dementia
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Amyotrophic lateral sclerosis-frontotemporal lobar dementia in 3 families with p.Ala382Thr TARDBP mutations.
|
20697052 |
2010 |
Presenile dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
We screened 37 AD, 8 mild cognitive impairment (MCI), 3 AD and CVD (cerebrovascular disease), 3 MCI and CVD, 8 frontotemporal dementia (FTD) and 2 progressive supranuclear palsy (PSP) patients, and 28 normal controls (NCs).We sequenced PSEN1, PSEN2 and APP (EOAD risk factors), as well as MAPT, GRN and TARDBP for all cases and NCs, and analysed the APOE, CLU, CR1 and PICALM genotypes as well as the MAPT and ACE haplotypes (LOAD risk factors) for the AD (n = 37) and AD + MCI (n = 45) cases and NCs (n = 28).We identified variants in PSEN1, PSEN2 and TARDBP across a range of phenotypes (AD, AD and CVD, FTD and PSP), suggesting that screening of all known candidate genes of Alzheimer's and non-Alzheimer's forms of dementias in all dementia cases might be warranted.
|
26159191 |
2015 |
Presenile dementia
|
0.100 |
Biomarker
|
disease |
BEFREE |
A recent paper reported that the TAR-DNA binding protein (TDP-43) is the disease protein in amyotrophic lateral sclerosis and frontotemporal lobar dementia with ubiquitin-positive inclusions.
|
19851068 |
2009 |