|
Huntington Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
In particular, we have previously shown that caspase cleavage of mutant HTT at amino acid position aspartate 586 (D586) by caspase-6 is critical for the pathogenesis of the disease in an HD mouse model.
|
30423259 |
2019 |
|
Huntington Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
The interaction of mHTT<sub>1-586</sub> with C6 may therefore promote a self-reinforcing, feedforward cycle of C6 zymogen activation and mHTT cleavage driving HD pathogenesis.
|
31353319 |
2019 |
|
Huntington Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
The Caspase-6 expressing mice and control littermates were subjected to behavioral tests to assess Huntington disease-relevant psychiatric, motor, and cognitive deficits.
|
29352267 |
2018 |
|
Huntington Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
Caspase-6 (CASP6) has an important role in axonal degeneration during neuronal apoptosis and in the neurodegenerative diseases Alzheimer and Huntington disease.
|
27911442 |
2017 |
|
Huntington Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
The activation of the aspartate-specific cysteinyl protease, Caspase-6, is proposed as an early pathogenic event of Alzheimer disease (AD) and Huntington's disease.
|
28241839 |
2017 |
|
Huntington Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
Since laquinimod is currently undergoing a clinical trial in Huntington disease (LEGATO-HD, clinicaltrials.gov ID: NCT02215616), we set out to evaluate its impact on neuronal caspase-6 activation.
|
27296315 |
2016 |
|
Huntington Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
Characterization of the interaction network provides important new information regarding key pathways of interactors of CASP6 and highlights potential novel therapeutic targets for HD, AD and cerebral ischemia.
|
26908611 |
2016 |
|
Huntington Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
Cleavage of the caspase-6 specific substrate lamin A is significantly increased in skeletal muscle obtained from HD patients as well as in muscle tissues from two different HD mouse models. p53, a transcriptional activator of caspase-6, is upregulated in neuronal cells and tissues expressing mutant huntingtin.
|
24070868 |
2014 |
|
Huntington Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
In particular, numerous findings implicate Caspase-6 (Casp6) in neurodegenerative diseases, including Alzheimer disease (AD) and Huntington disease (HD), highlighting the need for a deeper understanding of Casp6 biology and its role in brain development.
|
22262731 |
2012 |
|
Huntington Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
In this scenario, mutant htt fragments derived from caspase 6, or possibly other proteases, could mediate HD pathogenesis via a 'hit and run' type of mechanism in which caspase-6, or other larger N-terminal fragments, mediate a neurotoxic process before being cleaved to a smaller fragment that accumulates pathologically.
|
21515588 |
2011 |
|
Huntington Disease
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Here we demonstrate that activation of casp6, and not caspase-3, is observed before onset of motor abnormalities in human and murine HD brain.
|
21068307 |
2010 |
|
Huntington Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
Cleavage of huntingtin by caspase-6 at amino acid 586 is a crucial event in the pathogenesis of HD.
|
18992820 |
2009 |
|
Huntington Disease
|
0.100 |
Biomarker
|
disease |
BEFREE |
These results are consistent with proteolysis of htt at the caspase-6 cleavage site being an important event in mediating neuronal dysfunction and neurodegeneration and highlight the significant role of htt proteolysis and excitotoxicity in HD.
|
16777606 |
2006 |