|
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 |
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 |
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 |
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 |
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 |
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 |
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 |
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 |
|
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 |
Population stratification may bias analysis of PGC-1α as a modifier of age at Huntington disease motor onset.
|
22825315 |
2012 |
|
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 |
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 |
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 |
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 |
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 |