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Related Concept Videos

Electron Transport Chain: Complex I and II01:46

Electron Transport Chain: Complex I and II

The mitochondrial electron transport chain (ETC) is the main energy generation system in the eukaryotic cells. However, mitochondria also produce cytotoxic reactive oxygen species (ROS) due to the large electron flow during oxidative phosphorylation. While Complex I is one of the primary sources of superoxide radicals, ROS production by Complex II is uncommon and may only be observed in cancer cells with mutated complexes.
ROS generation is regulated and maintained at moderate levels necessary...
Animal Mitochondrial Genetics02:59

Animal 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...
ATP Synthase: Mechanism01:48

ATP 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...

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Related Experiment Video

Updated: Jun 28, 2026

Using Live Cell STED Imaging to Visualize Mitochondrial Inner Membrane Ultrastructure in Neuronal Cell Models
08:48

Using Live Cell STED Imaging to Visualize Mitochondrial Inner Membrane Ultrastructure in Neuronal Cell Models

Published on: June 30, 2023

Targeting mtDNA to Modulate Mitochondrial Dysfunction in Neurodegenerative Diseases.

Sanket Pramanik1, Biplab Debnath1, Aganta Chakraborty1

  • 1Department of Pharmaceutical Technology, Bharat Technology, Uluberia, 711316, West Bengal, India.

Molecular Neurobiology
|June 26, 2026
PubMed
Summary
This summary is machine-generated.

Mitochondrial DNA (mtDNA) damage contributes to neurodegenerative diseases. mtDNA-editing tools offer promising adjunctive strategies, but challenges remain for widespread therapeutic application.

Keywords:
DdCBE (DddA-derived Cytosine Base Editors)Heteroplasmy CorrectionMitoTALENsMitochondria-Targeted CRISPR/Cas SystemsMitochondrial Genome EditingNeurodegenerative DisordersOxidative Stress & Mitochondrial DysfunctionPrecision Medicine

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Modeling Mitochondrial Disease Using Brain Organoids: A Focus on Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like Episodes
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Published on: October 10, 2025

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Last Updated: Jun 28, 2026

Using Live Cell STED Imaging to Visualize Mitochondrial Inner Membrane Ultrastructure in Neuronal Cell Models
08:48

Using Live Cell STED Imaging to Visualize Mitochondrial Inner Membrane Ultrastructure in Neuronal Cell Models

Published on: June 30, 2023

Modeling Mitochondrial Disease Using Brain Organoids: A Focus on Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like Episodes
08:56

Modeling Mitochondrial Disease Using Brain Organoids: A Focus on Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like Episodes

Published on: October 10, 2025

Area of Science:

  • Neuroscience
  • Genetics
  • Molecular Biology

Background:

  • Mitochondrial dysfunction is a hallmark of neurodegenerative diseases like Alzheimer's and Parkinson's.
  • Secondary mitochondrial DNA (mtDNA) damage and heteroplasmy shifts can worsen neuronal vulnerability.

Purpose of the Study:

  • To review mitochondrial dysfunction in neurodegenerative diseases.
  • To evaluate current and emerging mtDNA editing techniques.
  • To highlight translational barriers for mtDNA-targeted therapies.

Main Methods:

  • Review of scientific literature on mitochondrial dysfunction and mtDNA editing.
  • Analysis of gene editing technologies including ZFNs, TALENs, DddA-base editors, and CRISPR/Cas systems.
  • Examination of limitations and challenges in mtDNA editing delivery and application.

Main Results:

  • mtDNA alterations exacerbate neurodegenerative disease pathology.
  • Various mtDNA editing platforms exist, each with unique strengths and limitations.
  • CRISPR/Cas systems face challenges with guiding RNA import into mitochondria.

Conclusions:

  • mtDNA-targeted interventions show potential as disease-modifying or adjunctive therapies.
  • These approaches are unlikely to be curative on their own.
  • Further research is needed to overcome delivery and specificity challenges in mtDNA editing.