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 |
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 |
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 |
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 |
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 |
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 |
RGD |
Metabolic and electrophysiological changes in the basal ganglia of transgenic Huntington's disease rats.
|
22813864 |
2012 |
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 |
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 |
LHGDN |
These results suggest that PGC-1alpha regulates multiple pathways in neurons and that HDACi's may be good candidates to target PGC-1alpha and GLUT4 in HD and other neurological disorders.
|
19118529 |
2009 |
Huntington Disease
|
0.300 |
Biomarker
|
disease |
BEFREE |
These results suggest that PGC-1alpha regulates multiple pathways in neurons and that HDACi's may be good candidates to target PGC-1alpha and GLUT4 in HD and other neurological disorders.
|
19118529 |
2009 |
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 |
Biomarker
|
disease |
BEFREE |
These studies suggest a key role for PGC-1alpha in the control of energy metabolism in the early stages of HD pathogenesis.
|
17018277 |
2006 |
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 |
Biomarker
|
disease |
BEFREE |
Ribosomal transcription is regulated by PGC-1alpha and disturbed in Huntington's disease.
|
28819135 |
2017 |
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 |
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 |
AlteredExpression
|
disease |
BEFREE |
Down-regulated expression of PGC-1alpha has been implicated in Huntington disease and in several Huntington disease animal models.
|
18090434 |
2008 |
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 |
Three new studies reveal that PGC-1alpha expression is downregulated in patients with Huntington's disease (HD) and in several animal models of this neurodegenerative disorder, implicating PGC-1alpha in HD pathogenesis and providing a connection between impaired energy metabolism and neurodegeneration.
|
17081970 |
2006 |
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 |
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 |
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 |
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 |