|
Lewy Body Disease
|
0.800 |
Biomarker
|
disease |
BEFREE |
We recently reported here that SNCA triplication results in a doubling in the amount of alpha-synuclein protein in blood from cases with hereditary Lewy body disease.
|
15663960 |
2005 |
|
Lewy Body Disease
|
0.800 |
Biomarker
|
disease |
BEFREE |
Increasing evidence suggests that α-synuclein (αS) aggregates in brains of individuals with Parkinson's disease and dementia with Lewy bodies can spread in a prion-like manner.
|
30030380 |
2018 |
|
Lewy Body Disease
|
0.800 |
Biomarker
|
disease |
BEFREE |
The interaction of α-synuclein with PSEN1 was detected in post-mortem brain tissue from cognitively normal cases and was significantly increased in tissue from cases with dementia with Lewy bodies and familial Alzheimer's disease associated with known PSEN1 mutations.
|
24860142 |
2014 |
|
Lewy Body Disease
|
0.800 |
Biomarker
|
disease |
BEFREE |
Our study suggests that catD upregulation may be an adaptive response to AD-related processes leading to neurofibrillary degeneration, but may not be directly associated with formation of α-synuclein inclusions in Lewy body dementia.
|
30051532 |
2019 |
|
Lewy Body Disease
|
0.800 |
Biomarker
|
disease |
BEFREE |
Yet to interfere with disease pathogenesis, we must dissect how small changes in αS homeostasis may give rise to Parkinson's disease (PD), dementia with Lewy bodies (DLB) and other human synucleinopathies.
|
30319334 |
2018 |
|
Lewy Body Disease
|
0.800 |
Biomarker
|
disease |
BEFREE |
Alpha-synuclein (aSyn) is the major protein component of Lewy bodies and Lewy neurites, the typical pathological hallmarks in Parkinson's disease (PD) and Dementia with Lewy bodies. aSyn is capable of inducing transcriptional deregulation, but the precise effect of specific aSyn mutants associated with familial forms of PD, remains unclear.
|
30092270 |
2018 |
|
Lewy Body Disease
|
0.800 |
AlteredExpression
|
disease |
BEFREE |
This study indicates that although levels of soluble α-synuclein protein are lower in DLB and PD, there is no evidence for a corresponding decrease in α-synuclein mRNA levels.
|
22560502 |
2012 |
|
Lewy Body Disease
|
0.800 |
Biomarker
|
disease |
BEFREE |
The patients with Parkinson's disease, Parkinson's disease with dementia, and dementia with Lewy bodies whose brain pathology indicated deposit of alpha-synuclein along with the co-occurrence of tau pathology and amyloid-beta plaques presented LRP10 mutations.
|
31582232 |
2019 |
|
Lewy Body Disease
|
0.800 |
Biomarker
|
disease |
BEFREE |
We evaluated the presence of phosphorylated α-synuclein in the retina of autopsied subjects with PD (9 subjects), incidental Lewy body disease (4 subjects), and controls (6 subjects) by immunohistochemistry and compared the retinal synucleinopathy with brain disease severity indicators.
|
29737566 |
2018 |
|
Lewy Body Disease
|
0.800 |
Biomarker
|
disease |
BEFREE |
Parkinson's disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA) are neurodegenerative disorders which have been pathologically classified as synucleinopathies, since they are associated with pathognomonic deposits of misfolded alpha-synuclein in cells of the nervous system.
|
29116402 |
2018 |
|
Lewy Body Disease
|
0.800 |
AlteredExpression
|
disease |
BEFREE |
Although the mechanisms through which glucocerebrosidase regulates the homeostasis of α-synuclein remains poorly understood, the identification of reduced glucocerebrosidase activity in the brains of patients with PD and dementia with Lewy bodies has paved the way for the development of novel therapeutic strategies directed at enhancing glucocerebrosidase activity and reducing α-synuclein burden, thereby slowing down or even preventing neuronal death.
|
30589955 |
2019 |
|
Lewy Body Disease
|
0.800 |
Biomarker
|
disease |
BEFREE |
Transgenic mice overexpressing tyrosine-to-cysteine mutant human alpha-synuclein: a progressive neurodegenerative model of diffuse Lewy body disease.
|
18238775 |
2008 |
|
Lewy Body Disease
|
0.800 |
AlteredExpression
|
disease |
BEFREE |
The present study explores alpha-synuclein 126 mRNA expression levels in the prefrontal cortex of six patients with dementia with Lewy bodies, eight patients with Lewy body variant of Alzheimer disease, eight patients with Alzheimer disease, and 10 controls.
|
16951579 |
2006 |
|
Lewy Body Disease
|
0.800 |
GeneticVariation
|
disease |
BEFREE |
Dementia with lewy bodies: GBA1 mutations are associated with cerebrospinal fluid alpha-synuclein profile.
|
31189032 |
2019 |
|
Lewy Body Disease
|
0.800 |
Biomarker
|
disease |
BEFREE |
Lewy body disease (LBD) is characterized by accumulation of aggregated α-synuclein in the central nervous system as eosinophilic cytoplasmic inclusions called Lewy bodies.
|
29909202 |
2018 |
|
Lewy Body Disease
|
0.800 |
Biomarker
|
disease |
BEFREE |
α-Synuclein is the most thoroughly investigated because of its close association with Parkinson's disease (PD), dementia with Lewy bodies and multiple system atrophy.
|
30650656 |
2019 |
|
Lewy Body Disease
|
0.800 |
GeneticVariation
|
disease |
BEFREE |
Mutations in SNCA predispose to Lewy-body dementia and cause Parkinson disease in humans and affect song production in songbirds.
|
29570792 |
2018 |
|
Lewy Body Disease
|
0.800 |
Biomarker
|
disease |
BEFREE |
Dementia with Lewy bodies (DLB) and Parkinson's Disease (PD) are neurodegenerative disorders of the aging population characterized by the abnormal accumulation of alpha-synuclein (alpha-syn).
|
21103359 |
2010 |
|
Lewy Body Disease
|
0.800 |
Biomarker
|
disease |
BEFREE |
Therefore, investigating the possible mechanism underlying αSyn aggregation is essential to understand the pathogenesis of DLB.
|
26515689 |
2016 |
|
Lewy Body Disease
|
0.800 |
Biomarker
|
disease |
BEFREE |
Alpha-synuclein (αS) is the major constituent of Lewy bodies and a pathogenic hallmark of all synucleinopathathies, including Parkinson's disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA).
|
31331359 |
2019 |
|
Lewy Body Disease
|
0.800 |
Biomarker
|
disease |
BEFREE |
Further, we demonstrate that αS aggregates isolated from postmortem human brains with diffuse Lewy body disease (DLBD) preferentially show endocytosis-mediated seeding associated with endo-lysosome rupture and have significantly reduced seeding activity compared to recombinant αS aggregates.
|
28794446 |
2017 |
|
Lewy Body Disease
|
0.800 |
Biomarker
|
disease |
BEFREE |
α-Synuclein dysfunction is the critical pathogenic event in Parkinson's disease, multiple system atrophy and dementia with Lewy bodies.
|
21863007 |
2011 |
|
Lewy Body Disease
|
0.800 |
Biomarker
|
disease |
BEFREE |
In the present study we 1) examined the molecular specificity of the novel anti-α-synuclein 5G4 antibody; 2) evaluated immunoreactivity patterns and their correlation in human brain tissue with micro- and astrogliosis in 57 cases with PD or DLB; and 3) performed a systematic immunoelectron microscopical mapping of subcellular localizations.
|
24878508 |
2014 |
|
Lewy Body Disease
|
0.800 |
GeneticVariation
|
disease |
BEFREE |
Heterozygous mutations in the lysosomal glucocerebrosidase gene (GBA1) have recently been recognized as the highest genetic risk factor for the development of α-synuclein aggregation disorders ("synucleinopathies"), including Parkinson's disease (PD) and dementia with Lewy bodies (DLB).
|
25573151 |
2015 |
|
Lewy Body Disease
|
0.800 |
Biomarker
|
disease |
BEFREE |
Neuropathological and genetic findings suggest that the presynaptic protein α-synuclein (aSyn) is involved in the pathogenesis of synucleinopathy disorders, including Parkinson's disease (PD), dementia with Lewy bodies (DLB) and multiple system atrophy.
|
28069058 |
2017 |