Mitochondrial Respiratory Chain

The mitochondrial respiratory chain is a series of electron-transfer complexes in the inner mitochondrial membrane that converts energy from nutrients into ATP, the cell’s primary energy currency. Electrons pass through complexes I, II, III, and IV via carriers such as coenzyme Q and cytochrome c, while complexes I, III, and IV pump protons into the intermembrane space; the resulting electrochemical gradient drives ATP synthase, with oxygen serving as the final electron acceptor and forming water. Studying this process helps explain cellular respiration, energy metabolism, reactive oxygen species production, and mitochondrial disorders, while supporting research into aging, metabolic disease, and potential therapeutic targets.

Mitochondrial Respiratory Chain - Related Videos

Research

JoVE EoE - Electrophoresis Techniques

Blue Native Polyacrylamide Gel Electrophoresis: A Non-Denaturing Separation Technique for Analysis of Intact Mitochondrial Respiratory Chain Complexes

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2025

In this video, we demonstrate an electrophoretic technique, called blue native PAGE, for the analysis of mitochondrial respiratory protein complexes in their natively folded and functionally active state. The anionic Coomassie blue dye causes the protein complexes to become negatively charged, allowing separation based on size and structure.

Research

JoVE Journal - Biochemistry
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Hybrid Clear/Blue Native Electrophoresis for the Separation and Analysis of Mitochondrial Respiratory Chain Supercomplexes

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Cited by 7 •

2019

Here we present a protocol to extract, resolve and identify mitochondrial supercomplexes which minimizes exposure to detergents and Coomassie Blue. This protocol offers an optimal balance between resolution, and preservation of enzyme activities, while minimizing the risk of losing labile protein-protein interactions.

Visualization of Mitochondrial Respiratory Function using Cytochrome C Oxidase / Succinate Dehydrogenase (COX/SDH) Double-labeling Histochemistry

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Cited by 82 •

2011

The cytochrome c oxidase/sodium dehydrogenase (COX/SDH) double-labeling method allows for direct visualization of mitochondrial respiratory enzyme deficiencies in fresh-frozen tissue sections. This is a straightforward histochemical technique and is useful in investigating mitochondrial diseases, aging, and aging-related disorders.

Analysis of the Expression and Complexes Assembly of the Mitochondrial Respiratory Chain Proteins in the Fission Yeast Schizosaccharomyces pombe

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2025

The fission yeast Schizosaccharomyces pombe is emerging as an attractive model for studying mitochondria. Here, we describe a protocol for analyzing the abundance and assembly of the mitochondrial respiratory complexes in S. pombe. This enables the characterization of conserved genes' novel functions in the mitochondrial respiratory chain.

Education

JoVE Core - Molecular Biology

Animal Mitochondrial Genetics

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2020

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...

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