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Related Concept Videos

Mitochondria01:37

Mitochondria

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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,...
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Animal Mitochondrial Genetics02:59

Animal Mitochondrial Genetics

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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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Electron Transport Chain: Complex I and II01:46

Electron Transport Chain: Complex I and II

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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...
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Mitochondrial Membranes01:45

Mitochondrial Membranes

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

Translocation of Proteins into the Mitochondria

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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,...
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ATP Synthase: Mechanism01:48

ATP Synthase: Mechanism

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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...
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Updated: Dec 29, 2025

Author Spotlight: Decoding Mitochondrial Aging
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Author Spotlight: Decoding Mitochondrial Aging

Published on: June 30, 2023

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Mitochondrial functions and rare diseases.

L Dard1, W Blanchard1, C Hubert2

  • 1Bordeaux University, 33000, Bordeaux, France; INSERM U1211, 33000, Bordeaux, France; CELLOMET, CGFB-146 Rue Léo Saignat, Bordeaux, France.

Molecular Aspects of Medicine
|February 8, 2020
PubMed
Summary
This summary is machine-generated.

Mitochondrial dysfunction, caused by genetic or environmental factors, is implicated in numerous rare diseases. This review surveys the biochemical, genetic, and clinical evidence, exploring diverse symptoms and therapeutic strategies.

Keywords:
BioenergeticsIatrogenyMitochondriaREDOXRare diseases

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Area of Science:

  • Cell Biology
  • Biochemistry
  • Genetics
  • Clinical Medicine

Background:

  • Mitochondria are vital organelles involved in energy production, signaling, biosynthesis, inflammation, and cell death.
  • Over 1100 human genes encode mitochondrial proteins, with defects linked to rare diseases.
  • Mitochondrial protein expression and function can be altered by genetic, epigenetic, and environmental factors.

Purpose of the Study:

  • To provide a comprehensive review of studies linking mitochondrial dysfunction to rare human diseases.
  • To discuss the diverse clinical manifestations of mitochondrial disorders.
  • To survey current and proposed therapeutic strategies, including clinical trials.

Main Methods:

  • Literature review of biochemical, genetic, and clinical studies.
  • Analysis of data from MITOCARTA database.
  • Survey of ongoing clinical trials for mitochondrial diseases.

Main Results:

  • Mitochondrial dysfunction is a significant factor in the pathogenesis of many rare diseases.
  • Symptoms associated with mitochondrial dysfunction exhibit considerable diversity.
  • Various therapeutic approaches are being investigated, with several clinical trials underway.

Conclusions:

  • Mitochondrial dysfunction plays a crucial role in a growing number of rare diseases.
  • Understanding these mechanisms is key to developing effective treatments.
  • Ongoing research and clinical trials offer hope for improved patient outcomes.