Search research articles

ABOUT JoVE

Overview Leadership Blog JoVE Help Center

AUTHORS

Publishing Process Editorial Board Scope & Policies Peer Review FAQ Submit

LIBRARIANS

Testimonials Subscriptions Access Resources Library Advisory Board FAQ

RESEARCH

JoVE Journal Methods Collections JoVE Encyclopedia of Experiments Archive

EDUCATION

JoVE Core JoVE Business JoVE Science Education JoVE Lab Manual Faculty Resource Center Faculty Site

Terms & Conditions of Use

Related Concept Videos

Translation

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...

Translation

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
Proteins are...

Translocation of Proteins into the Mitochondria

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,...

ATP Synthase: Mechanism

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...

Animal Mitochondrial Genetics

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 Sorting

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...

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by

Same author

Novel Clinical and Genetic Findings in Laurin-Sandrow Syndrome: A Case Report.

Molecular syndromology·2026

Same author

Dual CRISPR/Cas9 correction of compound heterozygous MARS2 mutations in the iPSC line ISMMSi060-A from a patient with COXPD25.

Stem cell research·2026

Same author

USP34 Haploinsufficiency as a Cause of Neurodevelopmental Phenotypes.

Clinical genetics·2026

Same author

Expanding Spectrum of FIG4-Related Neurological Disorders of Lysosomal Homeostasis: Case Report and Overview of the Potential Genotype-Phenotype Correlations.

Clinical genetics·2026

Same author

Characterizing the frequency of clinical events and assessing biomarkers in propionic acidemia: a natural history study.

Orphanet journal of rare diseases·2026

Same author

Generation of the induced pluripotent stem cell line ISMMSi061-A from a patient with ataxia, intention tremor, and hypotonia syndrome, childhood-onset.

Stem cell research·2026

Search research articles

Related Experiment Video

Updated: Nov 22, 2025

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

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

Published on: April 11, 2021

Mitochondrial translation defects and human disease.

Bryn D Webb¹, George A Diaz¹, Pankaj Prasun¹

¹Department of Genetics & Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA.

Journal of Translational Genetics and Genomics

|January 11, 2021

Summary

Mitochondrial translation is crucial for cellular energy production. Defects in this process, caused by mutations in mitochondrial genes, lead to various human diseases, particularly affecting high-energy organs.

Keywords:

Mitochondria aminoacyl-tRNA synthetase mitochondrial disease mtDNA rRNA ribosomal protein tRNA translation defect

More Related Videos

Author Spotlight: Advancing Techniques and Discoveries in Protein Synthesis and Assembly Through Innovative Mitochondrial Research

Author Spotlight: Advancing Techniques and Discoveries in Protein Synthesis and Assembly Through Innovative Mitochondrial Research

Published on: June 7, 2024

Author Spotlight: Decoding Mitochondrial Aging

Author Spotlight: Decoding Mitochondrial Aging

Published on: June 30, 2023

Related Experiment Videos

Last Updated: Nov 22, 2025

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

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

Published on: April 11, 2021

Author Spotlight: Advancing Techniques and Discoveries in Protein Synthesis and Assembly Through Innovative Mitochondrial Research

Author Spotlight: Advancing Techniques and Discoveries in Protein Synthesis and Assembly Through Innovative Mitochondrial Research

Published on: June 7, 2024

Author Spotlight: Decoding Mitochondrial Aging

Author Spotlight: Decoding Mitochondrial Aging

Published on: June 30, 2023

Area of Science:

Cellular Biology
Genetics
Biochemistry

Background:

Mitochondria generate cellular energy (ATP) through oxidative phosphorylation.
This process relies on 13 protein subunits encoded by mitochondrial DNA (mtDNA).
Mitochondrial translation is essential for synthesizing these mtDNA-encoded proteins.

Purpose of the Study:

To review diseases caused by defective mitochondrial translation.
To highlight the impact of mitochondrial genetic defects on cellular function.
To categorize disorders related to mitochondrial gene expression.

Main Methods:

Review of scientific literature on mitochondrial translation and associated diseases.
Analysis of genetic defects affecting mitochondrial protein synthesis.
Categorization of diseases based on affected components of the mitochondrial translation machinery.

Main Results:

Defects in mitochondrial translation lead to a spectrum of human diseases.
High-energy consuming organs are often most severely affected.
Specific disorders reviewed include those with mutations in mitochondrial tRNA, aminoacyl-tRNA synthetases, rRNA, and ribosomal proteins.

Conclusions:

Mitochondrial translation is vital for maintaining cellular energy homeostasis.
Disruptions in mitochondrial gene expression result in significant clinical pathologies.
Understanding these defects is key to diagnosing and potentially treating mitochondrial diseases.