Huntington Disease
|
0.300 |
Biomarker
|
disease |
BEFREE |
PGC-1α, Sirtuins and PARPs in Huntington's Disease and Other Neurodegenerative Conditions: NAD+ to Rule Them All.
|
31065944 |
2019 |
Huntington Disease
|
0.300 |
Biomarker
|
disease |
BEFREE |
Based on previous reports and our own research, we discuss in this review the potential role of HSF1 in mediating mitochondrial dysfunction in HD and propose a unifying mechanism that integrates the responses mediated by p53 and PGC-1α in HD <i>via</i> HSF1.
|
30941017 |
2019 |
Huntington Disease
|
0.300 |
AlteredExpression
|
disease |
BEFREE |
Recent findings have demonstrated that upregulation of PGC-1α expression in neurons can modulate MQC to prevent mitochondrial dysfunction in certain in vivo and in vitro aging or neurodegenerative encephalopathy models, such as Huntington's disease, Alzheimer's disease, and Parkinson's disease.
|
31410709 |
2019 |
Huntington Disease
|
0.300 |
Biomarker
|
disease |
BEFREE |
Our study strongly suggests that PGC-1α, as a master coregulator of mitochondrial biogenesis, energy homeostasis, and antioxidant defense, is a potential therapeutic target in HD.
|
30362565 |
2019 |
Huntington Disease
|
0.300 |
AlteredExpression
|
disease |
BEFREE |
Expression of mHTT in differentiated astrocytes induced cytosolic mHTT aggregates and nuclear inclusions, suppressed the expression of SOD2 and PGC1, reduced ability to uptake glutamate, decreased 4-aminopyridine (4-AP) response, and shifted I/V plot measured by electrophysiology, which are consistent with previous reports on HD astrocytes and patient brain samples.
|
30897183 |
2019 |
Huntington Disease
|
0.300 |
AlteredExpression
|
disease |
BEFREE |
To examine the effectiveness of βL on HD, βL was orally applied to R6/2 HD mice and behavioral phenotypes associated with HD, such as impairment of rota-rod performance and increase of clasping behavior, as well as changes of Sirt1 expression, CREB phosphorylation and PGC-1α deacetylation were examined.
|
29742127 |
2018 |
Huntington Disease
|
0.300 |
GeneticVariation
|
disease |
BEFREE |
Understanding the additive effects of PGC-1α gene functional variation and mutant huntingtin on transcription in this cell type may provide insight into the selective vulnerability of MSNs in HD.<b>SIGNIFICANCE STATEMENT</b> Reductions in peroxisome proliferator-activated receptor gamma coactivator-1α (PGC-1α)-mediated transcription have been implicated in the pathogenesis of Huntington's disease (<i>H</i>D).
|
29491012 |
2018 |
Huntington Disease
|
0.300 |
AlteredExpression
|
disease |
BEFREE |
By using multivariate logistic regression analysis, it was found that down-regulated PGC-1α expression is independently associated with the development of CVD in HD patients.
|
28550474 |
2017 |
Huntington Disease
|
0.300 |
Biomarker
|
disease |
BEFREE |
Ribosomal transcription is regulated by PGC-1alpha and disturbed in Huntington's disease.
|
28819135 |
2017 |
Huntington Disease
|
0.300 |
Biomarker
|
disease |
BEFREE |
SIRT3 expression depends on the activity of the metabolic master regulator peroxisome proliferator-activated receptor gamma coactivator 1-alpha (PGC-1α), a modifier of ALS and HD in patients and model organisms.
|
28603486 |
2017 |
Huntington Disease
|
0.300 |
AlteredExpression
|
disease |
BEFREE |
Recently, we showed that the administration of bezafibrate, a pan-PPAR agonist, increases the expression of PGC-1α and mitochondrial biogenesis, and improves phenotype and survival in R6/2 transgenic mouse model of HD.
|
27008868 |
2016 |
Huntington Disease
|
0.300 |
AlteredExpression
|
disease |
BEFREE |
PGC-1α mRNA levels are reduced in a number of neurodegenerative diseases and contribute to disease pathogenesis, since increased levels ameliorate behavioral defects and neuropathology of Huntington's disease, Parkinson's disease, and amyotrophic lateral sclerosis.
|
24398293 |
2014 |
Huntington Disease
|
0.300 |
Biomarker
|
disease |
BEFREE |
These results further strengthen the evidence for a role of PGC-1α in HD and unexpectedly suggest a gender effect.
|
24383721 |
2014 |
Huntington Disease
|
0.300 |
AlteredExpression
|
disease |
BEFREE |
RSG also prevented PGC-1α reduction and increased Sirt6 protein levels in HD mouse brain.
|
23373812 |
2013 |
Huntington Disease
|
0.300 |
GeneticVariation
|
disease |
BEFREE |
Several single-nucleotide polymorphisms (SNPs) located in two separate haplotype blocks of PPARGC1A have shown associations with Huntington's disease (HD) and Parkinson's disease, but causative SNPs have not been identified.
|
22589246 |
2012 |
Huntington Disease
|
0.300 |
Biomarker
|
disease |
RGD |
Metabolic and electrophysiological changes in the basal ganglia of transgenic Huntington's disease rats.
|
22813864 |
2012 |
Huntington Disease
|
0.300 |
AlteredExpression
|
disease |
BEFREE |
In 2006, a series of studies revealed that PGC-1α transcription interference contributes to HD neurodegeneration, linking the nuclear transcriptionopathy with the mitochondrial dysfunction.
|
22100502 |
2012 |
Huntington Disease
|
0.300 |
Biomarker
|
disease |
BEFREE |
Population stratification may bias analysis of PGC-1α as a modifier of age at Huntington disease motor onset.
|
22825315 |
2012 |
Huntington Disease
|
0.300 |
AlteredExpression
|
disease |
BEFREE |
Nicotinamide improves motor deficits and upregulates PGC-1α and BDNF gene expression in a mouse model of Huntington's disease.
|
20736066 |
2011 |
Huntington Disease
|
0.300 |
Therapeutic
|
disease |
RGD |
Mitogen- and stress-activated protein kinase 1-induced neuroprotection in Huntington's disease: role on chromatin remodeling at the PGC-1-alpha promoter.
|
21493629 |
2011 |
Huntington Disease
|
0.300 |
Biomarker
|
disease |
BEFREE |
Impaired activation of PGC-1alpha, therefore, plays an important role in the behavioral phenotype, metabolic disturbances and pathology of HD, which suggests the possibility that agents that enhance PGC-1alpha function will exert therapeutic benefits in HD patients.
|
20529956 |
2010 |
Huntington Disease
|
0.300 |
GeneticVariation
|
disease |
BEFREE |
In combination with PGC-1 alpha genotypes, 3.8% additional residual variance in HD AO can be explained.
|
20108082 |
2010 |
Huntington Disease
|
0.300 |
Biomarker
|
disease |
BEFREE |
The present review provides an overview of the advances in the understanding of the role of the PGC-1α system in HD pathogenesis and explores the implications for ALS, AD and PD.
|
20840068 |
2010 |
Huntington Disease
|
0.300 |
Biomarker
|
disease |
BEFREE |
Transcriptional processes regulated by peroxisome proliferator-activated receptor gamma (PPAR gamma) coactivator-1 alpha (PGC-1 alpha), which are critical for mitochondrial biogenesis, have been shown to be impaired in HD.
|
20556492 |
2010 |
Huntington Disease
|
0.300 |
Biomarker
|
disease |
BEFREE |
These findings show that impaired function of PGC-1alpha plays a critical role in muscle dysfunction in HD, and that treatment with agents to enhance PGC-1alpha function could exert therapeutic benefits.
|
19460884 |
2009 |