|
Huntington Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
In the central nervous system (CNS), EVs have been suggested as potential carriers in the intercellular delivery of misfolded proteins associated to neurodegenerative disorders, such as tau and amyloid β in Alzheimer's Disease (AD), α-synuclein in Parkinson's Disease (PD), superoxide dismutase (SOD)1 in amyotrophic lateral sclerosis and huntingtin in Huntington's Disease.
|
29305855 |
2018 |
|
Huntington Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
Also the role of GAPDH in neurodegenerative diseases is linked to it directly binding to specific amyloidogenic proteins and petides such as β-amyloid precursor protein, β-amyloid peptide and tau protein in Alzheimer's disease, huntingtin in Huntington's disease and α-synuclein in Parkinson disease.
|
30254002 |
2018 |
|
Huntington Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
Finally, following formic acid extraction, we observed increased aggregation of TDP-43, α-Syn, and phosphorylated-Tau during HD progression.
|
29134321 |
2018 |
|
Huntington Disease
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Here, we describe a sensitive fluorescence-based method to observe, monitor, and quantify mild Drosophila eye degeneration caused by various proteins, including the polyglutamine disease proteins ataxin-3 (spinocerebellar ataxia type 3) and huntingtin (Huntington's disease), mutant α-synuclein (Parkinson's disease), and Aβ42 (Alzheimer's disease).
|
24798551 |
2014 |
|
Huntington Disease
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Further investigation showed that among three single components derived from Radix Polygalae--i.e., polygalacic acid, senegenin and onjisaponin B--onjisaponin B was able to induce autophagy and accelerate both the removal of mutant huntingtin and A53T α-synuclein, which are highly associated with Huntington disease and Parkinson disease, respectively.
|
24248062 |
2013 |
|
Huntington Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
Here, we used a bimolecular fluorescence complementation assay to study the initial steps of the co-aggregation of huntingtin (Htt) and α-synuclein (α-syn), two aggregation-prone proteins involved in Huntington's disease (HD) and Parkinson's disease (PD), respectively.
|
22119730 |
2012 |
|
Huntington Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
α-Synuclein accumulates in huntingtin inclusions but forms independent filaments and its deficiency attenuates early phenotype in a mouse model of Huntington's disease.
|
22045698 |
2012 |
|
Huntington Disease
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
On the other hand, α-synuclein deletion in both HD models increases autophagosome numbers and this is associated with a delayed onset of tremors and weight loss, two of the most prominent endophenotypes of the HD-like disease in mice.
|
22010050 |
2012 |
|
Huntington Disease
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Prion protein (Prion diseases), amyloid-beta (Alzheimer's disease), alpha-synuclein (Parkinson's disease), Huntingtin (Huntington's disease), serum amyloid A (AA amyloidosis) and islet amyloid polypeptide (type 2 diabetes) are some of the proteins that trigger disease when they get misfolded.
|
21571086 |
2011 |
|
Huntington Disease
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
In yeast models of Huntington's disease, Hsp104 is required for the aggregation and toxicity of polyglutamine (polyQ), but the expression of Hsp104 in cellular and animal models of Huntington's and Parkinson's disease protects against polyQ and alpha-synuclein toxicity.
|
19768774 |
2010 |
|
Huntington Disease
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Trehalose-induced autophagy enhanced the clearance of autophagy substrates like mutant huntingtin and the A30P and A53T mutants of alpha-synuclein, associated with Huntington disease (HD) and Parkinson disease (PD), respectively.
|
17182613 |
2007 |
|
Huntington Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
Misfolded proteins accumulate in many neurodegenerative diseases, including huntingtin in Huntington's disease and alpha-synuclein in Parkinson's disease.
|
16537516 |
2006 |
|
Huntington Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
The followings, for instance, are only a few of the many new biomarkers that have been recently identified: the phosphorylated tau protein and aggregated Beta-amyloid peptide for Alzheimer's disease (AD), Alpha-synuclein contained Lewy bodies and altered dopamine transporter (DAT) imaging for Parkinson's disease (PD), SOD mutations for familial amyotrophic lateral sclerosis (ALS), and CAG repeats resulted from Huntington's gene mutations in Huntington's disease (HD).
|
15538967 |
2004 |
|
Huntington Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
Lentiviral vectors also provide a new strategy for in vivo modeling of human diseases; for example, the lentiviral-mediated overexpression of mutated human alpha-synuclein or huntingtin genes in basal ganglia induces neuronal pathology in animals resembling PD and HD in man.
|
15352068 |
2004 |
|
Huntington Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
These 33 nontau FTLD cases, along with five Alzheimer's disease (AD) and six Huntington's disease (HD) cases with severe pathology, showed a variable loss of soluble tau proteins, broadly comparable with the extent of neuronal loss from the cortex and loss of the intracortical perikaryal marker, NeuN, but unrelated to proteins within afferent projection fibres such as neurofilament and alpha-synuclein.
|
14720172 |
2004 |