Amyotrophic Lateral Sclerosis
|
0.490 |
Biomarker
|
disease |
BEFREE |
Finally, we highlight recent progress in research on the involvement of PGC-1α in certain major neurodegenerative disorders (NDDs), including Alzheimer's disease, Parkinson's disease, Huntington's disease, and amyotrophic lateral sclerosis.
|
29580918 |
2018 |
Amyotrophic Lateral Sclerosis
|
0.490 |
Biomarker
|
disease |
BEFREE |
Moreover, PGC-1α, a disease modifier of ALS, was restored by CysC through AMP-activated kinase activation.
|
29282717 |
2018 |
Amyotrophic Lateral Sclerosis
|
0.490 |
Biomarker
|
disease |
BEFREE |
Based on these findings we conclude that SIRT3 mediates the different effects of PGC-1α during the course of transgenic (tg) ALS and HD and in the human conditions only partial aspects Sirt3 dysregulation manifest.
|
28603486 |
2017 |
Amyotrophic Lateral Sclerosis
|
0.490 |
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 |
Amyotrophic Lateral Sclerosis
|
0.490 |
GermlineModifyingMutation
|
disease |
ORPHANET |
PGC-1α is a male-specific disease modifier of human and experimental amyotrophic lateral sclerosis.
|
23669350 |
2013 |
Amyotrophic Lateral Sclerosis
|
0.490 |
Biomarker
|
disease |
BEFREE |
In complementary animal studies, we show that deficiency of full-length (FL) Pgc-1α leads to a significantly earlier age of onset and a borderline shortened survival in male, but not in female ALS-transgenic mice.
|
23669350 |
2013 |
Amyotrophic Lateral Sclerosis
|
0.490 |
AlteredExpression
|
disease |
BEFREE |
As miR-23a negatively regulates PGC-1α signalling, therapeutic inhibition of miR-23a may be a strategy to rescue PGC-1α activity and ameliorate skeletal muscle mitochondrial function in ALS.
|
22975021 |
2013 |
Amyotrophic Lateral Sclerosis
|
0.490 |
AlteredExpression
|
disease |
BEFREE |
We investigated mRNA and protein expressions of PGC-1α and PGC-1α-regulated factors in the spinal cord and muscle tissues of SOD1 ALS mice and in ALS patients.
|
23147503 |
2012 |
Amyotrophic Lateral Sclerosis
|
0.490 |
AlteredExpression
|
disease |
BEFREE |
Therefore, muscle is not a primary target of mutant SOD1-mediated toxicity, but drugs increasing PGC-1α activity in muscle represent an attractive therapy for maintaining muscle function during progression of ALS.
|
22560226 |
2012 |
Amyotrophic Lateral Sclerosis
|
0.490 |
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 |
Amyotrophic Lateral Sclerosis
|
0.490 |
GeneticVariation
|
disease |
GWASDB |
Genome-wide genotyping in amyotrophic lateral sclerosis and neurologically normal controls: first stage analysis and public release of data.
|
17362836 |
2007 |
Amyotrophic Lateral Sclerosis
|
0.490 |
Biomarker
|
disease |
HPO |
|
|
|