Related Experiment Video
Updated: May 12, 2026
08:48
Using Live Cell STED Imaging to Visualize Mitochondrial Inner Membrane Ultrastructure in Neuronal Cell Models
Published on: June 30, 2023
PGC-1α, mitochondrial dysfunction, and Huntington's disease
Ashu Johri1, Abhishek Chandra1, M Flint Beal1
1Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York-Presbyterian Hospital, New York, NY 10065, USA.
Free Radical Biology & Medicine
|April 23, 2013
Summary
Huntington's disease (HD) involves mitochondrial dysfunction, partly due to impaired peroxisome proliferator-activated receptor gamma coactivator 1-alpha (PGC-1α). Targeting PGC-1α may offer a therapeutic strategy for HD.
Area of Science:
- Neuroscience
- Mitochondrial Biology
- Genetics
Background:
- Neurons have high energy demands, relying on mitochondria.
- Mitochondrial dysfunction impairs ATP production, calcium buffering, and increases reactive oxygen species.
- Mitochondrial dysfunction is linked to neurodegeneration and Huntington's disease (HD) pathogenesis.
Purpose of the Study:
- To review key findings on the role of PGC-1α in HD-related mitochondrial dysfunction.
- To explore PGC-1α as a potential therapeutic target for HD.
Main Methods:
- Literature review and synthesis of existing research findings.
- Focus on studies implicating PGC-1α in mitochondrial dysfunction in HD.
Main Results:
- Impaired function of PGC-1α is implicated in mitochondrial dysfunction in HD.
- PGC-1α is a key regulator of mitochondrial biogenesis, metabolism, and antioxidant defenses.
Conclusions:
- PGC-1α plays a critical role in mitochondrial health within the context of HD.
- Modulating PGC-1α activity presents a promising therapeutic avenue for treating Huntington's disease.
Keywords:
3-NP3-nitropropionic acid4-hydroxynonenal8-OHdG8-hydroxy-2′-deoxyguanosineBATEnergy metabolismHDHNEHuntington's diseaseMBPMDAMitochondrial dynamicsMitochondrial dysfunctionMitophagyNOXNADPHNeurodegenerationOXPHOSOxidative stressPGC-1αPPARPPARsROSSIRT1SIRT3Therapeuticsbrown adipose tissuemalondialdehydemyelin basic proteinoxidaseoxidative phosphorylationperoxisome proliferator-activated receptorperoxisome proliferator-activated receptor-γ coactivator-1αreactive oxygen species
Related Concept Videos
01:21Huntington Disease l: Introduction
Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show reduced penetrance,...
01:24Parkinson Disease ll: Pathophysiology
Parkinson disease (PD) is a progressive neurodegenerative disorder primarily affecting movement, with additional non-motor features. Its pathophysiology involves complex interactions among genetic susceptibility, environmental exposures, and cellular dysfunction, including dopaminergic neuron loss, protein aggregation, and mitochondrial impairment.Selective NeurodegenerationA key feature is the degeneration of dopaminergic neurons in the substantia nigra pars compacta, leading to reduced...
01:24Parkinson Disease l: Introduction
Parkinson’s disease is a chronic, progressive neurodegenerative disorder that primarily affects movement. It is characterized by motor symptoms such as resting tremors, muscle rigidity, bradykinesia (slowness of movement), and postural instability. Patients may notice hand tremors at rest, stiffness during movement, or a shuffling gait. In addition to motor features, non-motor symptoms include sleep disturbances, mood and behavioral changes, constipation, and cognitive impairment, all of which...
01:48ATP Synthase: Mechanism
In animals, the mitochondrial F1F0 ATP synthase is the key protein that synthesizes ATP molecules through a complex catalytic mechanism. While the nuclear genome encodes the majority of ATP synthase subunits, the mitochondrial genome encodes some of the enzyme's most critical components. The formation of this multi-subunit enzyme is a complex multi-step process regulated at the level of transcription, translation, and assembly. Defects in one or more of these steps can result in decreased ATP...
01:37Mitochondria
Mitochondria are eukaryotic cellular organelles that are known to produce energy through a process called oxidative phosphorylation. Besides their primary function, mitochondria are involved in various cellular processes, including cell growth, differentiation, signaling, metabolism, and senescence. Age-related changes cause a decline in mitochondrial quality and integrity due to increased mitochondrial mutations and oxidative damage. Thus, aging can severely impact mitochondrial functions,...
02:59Animal Mitochondrial Genetics
Among all the organelles in an animal cell, only mitochondria have their own independent genomes. Animal mitochondrial DNA is a double-stranded, closed-circular molecule with around 20,000 base pairs. Mitochondrial DNA is unique in that one of its two strands, the heavy, or H, -strand is guanine rich, whereas the complementary strand is cytosine rich and called the light, or L, -strand. Compared to nuclear DNA, mitochondrial DNA has a very low percentage of non-coding regions and is marked by...