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Related Concept Videos

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...
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...
Translocation of Proteins into the Mitochondria01:19

Translocation of Proteins into the Mitochondria

Mitochondrial precursors are translocated to the internal subcompartments via independent mechanisms involving distinct protein machineries called translocases.
Sorting of outer membrane proteins:
Mitochondrial outer membrane proteins are of two types: the transmembrane, beta-barrel porins, and the membrane-anchored, alpha-helical proteins. Beta-barrel porin precursors are translocated by the TOM complex and inserted into the outer mitochondrial membrane by the SAM complex. In contrast,...
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...
Energy to Drive Translocation01:37

Energy to Drive Translocation

Mitochondrial protein import is powered by two distinct energy sources: ATP hydrolysis and electrochemical potential across the inner membrane. Newly synthesized precursors are bound by cytosolic chaperones of the Hsp70 family, which guide them to the import receptors on the mitochondrial surface. Utilizing the energy of ATP hydrolysis, Hsp70 chaperones transfer these precursors to the TOM receptors on the mitochondrial outer membrane.
Generally, polypeptides are unfolded by two distinct...
Translation01:31

Translation

Lesson: Translation
Translation is the process of synthesizing proteins from the genetic information carried by messenger RNA (mRNA). Following transcription, it constitutes the final step in the expression of genes. This process is carried out by ribosomes, complexes of protein and specialized RNA molecules. Ribosomes, transfer RNA (tRNA), and other proteins produce a chain of amino acids—the polypeptide—as the end product of translation.
Translation Produces the Building Blocks of Life

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Related Experiment Video

Updated: May 24, 2026

Analyzing Oxygen Consumption Rate in Primary Cultured Mouse Neonatal Cardiomyocytes Using an Extracellular Flux Analyzer
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Analyzing Oxygen Consumption Rate in Primary Cultured Mouse Neonatal Cardiomyocytes Using an Extracellular Flux Analyzer

Published on: February 13, 2019

Disruption of ATP-binding cassette B8 in mice leads to cardiomyopathy through a decrease in mitochondrial iron

Yoshihiko Ichikawa1, Marina Bayeva, Mohsen Ghanefar

  • 1Feinberg Cardiovascular Research Institute, Northwestern University School of Medicine, Chicago, IL 60611, USA.

Proceedings of the National Academy of Sciences of the United States of America
|March 1, 2012
PubMed
Summary

The ATP-binding cassette (ABC) transporter ABCB8 is crucial for exporting iron from mitochondria. Its absence causes iron overload, heart damage, and impaired cytosolic iron-sulfur protein maturation.

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Last Updated: May 24, 2026

Analyzing Oxygen Consumption Rate in Primary Cultured Mouse Neonatal Cardiomyocytes Using an Extracellular Flux Analyzer
11:26

Analyzing Oxygen Consumption Rate in Primary Cultured Mouse Neonatal Cardiomyocytes Using an Extracellular Flux Analyzer

Published on: February 13, 2019

Phosphorus-31 Magnetic Resonance Spectroscopy: A Tool for Measuring In Vivo Mitochondrial Oxidative Phosphorylation Capacity in Human Skeletal Muscle
09:40

Phosphorus-31 Magnetic Resonance Spectroscopy: A Tool for Measuring In Vivo Mitochondrial Oxidative Phosphorylation Capacity in Human Skeletal Muscle

Published on: January 19, 2017

Area of Science:

  • Cell Biology
  • Biochemistry
  • Cardiovascular Research

Background:

  • Mitochondrial iron homeostasis is vital for cellular function, balancing iron's role in heme and Fe/S cluster synthesis against its potential for oxidative stress.
  • The ATP-binding cassette (ABC) transporter ABCB8, located in the mitochondrial inner membrane, has an uncharacterized function.

Purpose of the Study:

  • To investigate the function of ABCB8 in mitochondrial iron regulation and its impact on cardiac health.
  • To determine ABCB8's role in the maturation of iron-sulfur proteins.

Main Methods:

  • Genetic deletion of ABCB8 in mouse hearts.
  • Echocardiography and invasive hemodynamics to assess cardiac function.
  • In vitro studies using isolated mitochondria to measure iron export.
  • Assessing activity of cytosolic and mitochondrial iron-sulfur enzymes.

Main Results:

  • Genetic deletion of ABCB8 in mouse hearts led to mitochondrial iron accumulation, cardiomyopathy, increased reactive oxygen species, and cell death.
  • In vitro, ABCB8 down-regulation decreased mitochondrial iron export, while overexpression increased it.
  • ABCB8 deletion impaired the maturation and activity of cytosolic iron-sulfur proteins, but not mitochondrial ones.

Conclusions:

  • ABCB8 is essential for mitochondrial iron export and maintaining cardiac function.
  • ABCB8 plays a critical role in mitochondrial iron homeostasis.
  • ABCB8 is required for the proper maturation of cytosolic iron-sulfur proteins.