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Related Concept Videos

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
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
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,...
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 Protein Sorting01:39

Mitochondrial Protein Sorting

Mitochondria are double-membrane organelles of the eukaryotes involved in cellular metabolism, signaling, ATP synthesis, and programmed cell death.  Each of these processes requires specific proteins and enzymes that must be correctly sorted to the right mitochondrial subcompartment for the proper functioning of the organelle.
Most of these mitochondrial proteins are encoded by the nucleus and imported to the mitochondria as unfolded or loosely folded precursors. Mitochondrial precursors...
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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Updated: May 18, 2026

Labelling and Visualization of Mitochondrial Genome Expression Products in Baker's Yeast Saccharomyces cerevisiae
08:33

Labelling and Visualization of Mitochondrial Genome Expression Products in Baker's Yeast Saccharomyces cerevisiae

Published on: April 11, 2021

Mitochondrial diseases: translation matters.

Sarah Pearce1, Catherine Laura Nezich, Antonella Spinazzola

  • 1MRC Mitochondrial Biology Unit, Wellcome Trust-MRC Building, Hills Road Cambridge, CB2 0XY, UK.

Molecular and Cellular Neurosciences
|September 19, 2012
PubMed
Summary
This summary is machine-generated.

Mitochondrial diseases can stem from impaired mitochondrial protein synthesis. Recent research reveals more neurological conditions linked to faulty mitochondrial translation machinery.

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Area of Science:

  • Biochemistry
  • Genetics
  • Neuroscience

Background:

  • Mitochondrial diseases are linked to energy production defects.
  • Defects in mitochondrial protein synthesis are a known cause.
  • The full scope of proteins involved in mitochondrial translation is now better understood.

Purpose of the Study:

  • To review the mechanisms and factors of mammalian mitochondrial translation.
  • To discuss pathologies arising from mitochondrial translation dysfunction.
  • To highlight the connection between mitochondrial translation and neurodegeneration.

Main Methods:

  • Literature review of current research on mitochondrial translation.
  • Analysis of genetic and molecular factors affecting mitochondrial protein synthesis.
  • Examination of disease-associated pathologies linked to mitochondrial dysfunction.

Main Results:

  • Mitochondrial translation involves a complex array of proteins.
  • Impairments at any stage of this process can lead to disease.
  • Numerous neurological and other disorders are now linked to impaired mitochondrial translation.

Conclusions:

  • Understanding mitochondrial translation is crucial for diagnosing and treating mitochondrial diseases.
  • Dysfunctional mitochondrial translation is a significant contributor to various pathologies, particularly neurodegeneration.
  • Further research into mitochondrial translation mechanisms will likely uncover more disease links.