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Using Live Cell STED Imaging to Visualize Mitochondrial Inner Membrane Ultrastructure in Neuronal Cell Models
Published on: June 30, 2023
Mitochondrial diseases of the brain
Rajnish K Chaturvedi1, M Flint Beal
1CSIR-Indian Institute of Toxicology Research, 80 MG Marg, Lucknow 226001, India. rajnish@iitr.res.in
Free Radical Biology & Medicine
|April 10, 2013
Summary
Mitochondrial dysfunction is a key factor in neurodegenerative disorders like Alzheimer's and Parkinson's. This review explores mitochondrial roles and therapeutic strategies, including bioenergetic agents and novel targets.
Area of Science:
- Neuroscience
- Cell Biology
- Genetics
Background:
- Neurodegenerative disorders cause significant cognitive, motor, and behavioral impairments.
- Mitochondrial dysfunction is increasingly recognized as a central pathogenic mechanism in these diseases.
- Specific disorders include Parkinson's disease, Alzheimer's disease, and Amyotrophic lateral sclerosis.
Purpose of the Study:
- To review the multifaceted roles of mitochondrial dysfunction in neurodegenerative diseases.
- To explore the therapeutic potential of targeting mitochondria and related pathways.
- To discuss current and future therapeutic strategies for these debilitating conditions.
Main Methods:
- Literature review focusing on mitochondrial dysfunction in neurodegenerative disorders.
- Analysis of studies on bioenergetic agents in animal models and clinical trials.
- Examination of novel therapeutic targets such as PGC-1α, TORC-1, AMP kinase, Nrf2-ARE, and Sirtuins.
Main Results:
- Mitochondrial dysfunction manifests as bioenergetics defects, DNA/gene mutations, altered dynamics, and impaired transcription.
- Mitochondrial dysfunction is implicated across a spectrum of neurodegenerative diseases.
- Bioenergetic agents show promise in preclinical models and are under investigation in clinical trials.
Conclusions:
- Mitochondrial dysfunction is a critical therapeutic target for neurodegenerative disorders.
- Targeting pathways like PGC-1α, TORC-1, AMP kinase, Nrf2-ARE, and Sirtuins offers novel treatment avenues.
- Further research into mitochondrial mechanisms and therapeutic interventions is crucial for combating neurodegeneration.
Keywords:
1-methyl-4-phenyl-1,2,3,6-tetrahydrodropyridine1-methyl-4-phenylpyridinium6-OHDA6-hydroxydopamineADALSAMP-activated protein kinaseAMPKAlzheimer’s diseaseAmyotrophic Lateral SclerosisAmyotrophic lateral sclerosisBATCKCharcot-Marie-Tooth disease and Friedreich’s ataxiaCo-Q10CoQ10CreatineDrp1Free radicalsHDHuntington’s DiseaseHuntington’s diseaseKGDHMFNMMPMPP+MPTPMitochondrial dysfunctionNeurodegenerative diseasesPCrPDPGC-1αPPARsParkinson’s diseaseROSSirtuinsTAR DNATCATORCTfamTransduceres of Creb-related binding proteinUCP-1alpha-ketoglutarate dehydrogenasebinding protein TDP-43brown adipose tissuecoenzymeQ10creatine kinasedynamin-related protein 1mitochondrial DNAmitochondrial membrane potentialmitochondrial transcription factor AmitofusinmtDNAperoxisome proliferator-activated receptorsphosphocreatineproliferator-activated receptor-gamma coactivator-1alphareactive oxygen speciestricarboxylic acid cycleuncoupling proteinsMore Related Videos
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