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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...
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...
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...
Mitochondrial Membranes01:45

Mitochondrial Membranes

A single mitochondrion is a bean-shaped organelle enclosed by a double-membrane system. The outer membrane of mitochondria is smooth and contains many porins - the integral membrane transporters. Porins enable free diffusion of ions and small uncharged molecules through the outer mitochondrial membrane but limit the transport of molecules larger than 5000 Daltons. Further, the outer mitochondrial membrane forms a unique structure called membrane contact sites with other subcellular organelles,...
Mitochondria01:37

Mitochondria

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,...
The Electron Transport Chain01:30

The Electron Transport Chain

The electron transport chain or oxidative phosphorylation is an exothermic process in which free energy released during electron transfer reactions is coupled to ATP synthesis. This process is a significant source of energy in aerobic cells, and therefore inhibitors of the electron transport chain can be detrimental to the cell's metabolic processes.
Inhibitors of the electron transport chain
Rotenone, a widely used pesticide, prevents electron transfer from Fe-S cluster to ubiquinone or Q in...

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Related Experiment Video

Updated: May 23, 2026

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

Treatment for mitochondrial disorders.

Gerald Pfeffer1, Kari Majamaa, Douglass M Turnbull

  • 1Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, UK.

The Cochrane Database of Systematic Reviews
|April 20, 2012
PubMed
Summary
This summary is machine-generated.

Current treatments for mitochondrial respiratory chain disorders lack clear evidence of efficacy. Further research is needed to identify effective interventions for these inherited neurometabolic diseases.

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Experimental Protocol for Detecting Mitochondrial Function in Hepatocytes Exposed to Organochlorine Pesticides
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An In Vitro Approach to Study Mitochondrial Dysfunction: A Cybrid Model
06:05

An In Vitro Approach to Study Mitochondrial Dysfunction: A Cybrid Model

Published on: March 9, 2022

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Last Updated: May 23, 2026

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

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Published on: October 10, 2025

Experimental Protocol for Detecting Mitochondrial Function in Hepatocytes Exposed to Organochlorine Pesticides
08:39

Experimental Protocol for Detecting Mitochondrial Function in Hepatocytes Exposed to Organochlorine Pesticides

Published on: September 16, 2020

An In Vitro Approach to Study Mitochondrial Dysfunction: A Cybrid Model
06:05

An In Vitro Approach to Study Mitochondrial Dysfunction: A Cybrid Model

Published on: March 9, 2022

Area of Science:

  • Neurology
  • Metabolic Disorders
  • Genetics

Background:

  • Mitochondrial respiratory chain disorders are common inherited neurometabolic diseases.
  • They cause neurological symptoms and multi-organ involvement, leading to significant morbidity and mortality.
  • Existing treatments are supportive, with unclear efficacy.

Purpose of the Study:

  • To evaluate objective evidence supporting current treatments for mitochondrial disease.
  • To identify new studies and assess the bias of existing clinical trials.

Main Methods:

  • Systematic review of randomized controlled trials (RCTs) and cross-over studies.
  • Searched multiple databases (Cochrane, CENTRAL, MEDLINE, EMBASE) and contacted experts.
  • Included participants with confirmed mitochondrial disease; interventions included pharmacological agents, supplements, and exercise therapy.

Main Results:

  • Twelve studies met inclusion criteria; eight were new since the prior review.
  • Comparability between studies was low due to variations in disease, agents, dosage, design, and outcomes.
  • No intervention demonstrated clear clinical benefit; some showed biochemical improvements or had adverse events (e.g., DCA toxicity).

Conclusions:

  • Despite new trials, no intervention currently has clear supporting evidence for mitochondrial disorders.
  • Further research is essential to establish the efficacy of various therapeutic approaches.
  • Future studies should focus on novel agents in homogeneous populations with clinically relevant endpoints.