Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Concept Videos

Animal Mitochondrial Genetics02:59

Animal Mitochondrial Genetics

9.0K
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...
9.0K
Translocation of Proteins into the Mitochondria01:19

Translocation of Proteins into the Mitochondria

12.1K
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,...
12.1K
Export of Mitochondrial and Chloroplast Genes02:19

Export of Mitochondrial and Chloroplast Genes

4.1K
A eukaryotic cell can have up to three different types of genetic systems: nuclear, mitochondrial, and chloroplast. During evolution, organelles have exported many genes to the nucleus; this transfer is still ongoing in some plant species. Approximately 18% of the Arabidopsis thaliana nuclear genome is thought to be derived from the chloroplast’s cyanobacterial ancestor, and around 75% of the yeast genome derived from the mitochondria’s bacterial ancestor. This export has occurred...
4.1K
Mitochondrial Precursor Proteins01:39

Mitochondrial Precursor Proteins

3.5K
Mitochondrial precursors are partially unfolded or loosely folded polypeptide chains. Newly synthesized precursors are inhibited from spontaneously folding into their native conformation by the cytosolic chaperones, heat shock proteins 70 (Hsp70), and mitochondrial import stimulation factors (MSFs). Precursors bound to MSFs are guided to the TOM70-TOM37 receptors, while precursors bound to Hsp70  chaperones are targetted to TOM20-TOM22 receptor complexes.
Most of the mitochondrial...
3.5K
Mitochondrial Protein Sorting01:39

Mitochondrial Protein Sorting

5.6K
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...
5.6K

You might also read

Related Articles

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

Sort by
Same author

Conserved and Lineage-Specific Roles of KEA-Mediated Ion Homeostasis in Chlamydomonas.

Plant physiology·2026
Same author

In the absence of mitochondrial fusion unequal segregation of mitochondria drives mtDNA loss.

EMBO reports·2026
Same author

mtHsp70 chaperone converts mitochondrial proteostasis stress into impaired protein import.

Proceedings of the National Academy of Sciences of the United States of America·2026
Same author

Mrx6 binds the Lon protease Pim1 N-terminal domain to confer selective substrate specificity and regulate mtDNA copy number.

Nucleic acids research·2026
Same author

Nanoscale dynamics of enhancer-promoter interactions during exit from pluripotency.

Nucleic acids research·2025
Same author

The origin of septin ring size control in budding yeast.

The EMBO journal·2025
Same journal

Genetic survey of biomarkers at early and mid-pregnancy identifies pregnancy-specialized immune regulation.

PLoS genetics·2026
Same journal

Argonaute proteins orchestrate Meiotic Sex Chromosome Inactivation and timing of the spermatogenic transcriptional program.

PLoS genetics·2026
Same journal

Genome wide association study meta-analysis of neuropathologic lesions of Alzheimer's disease and related dementias in a multi-site autopsy cohort.

PLoS genetics·2026
Same journal

Microtubule stiffening by the doublecortin-domain protein ZYG-8 contributes to mitotic spindle orientation during zygote division in Caenorhabditis elegans.

PLoS genetics·2026
Same journal

Multiple instance fine-mapping: Predicting causal regulatory variants with a deep sequence model.

PLoS genetics·2026
Same journal

Nuclear ubiquitin-conjugating enzyme TrUbc4 and F-box protein TrFwd1-mediated modification of Cre1 in Trichoderma reesei establishes a regulatory mechanism for carbon catabolite repression.

PLoS genetics·2026
See all related articles

Related Experiment Video

Updated: Jan 13, 2026

Author Spotlight: High-Throughput Image-Based Quantification of Mitochondrial DNA Synthesis and Distribution
10:47

Author Spotlight: High-Throughput Image-Based Quantification of Mitochondrial DNA Synthesis and Distribution

Published on: May 5, 2023

4.4K

Local mitochondrial physiology defined by mtDNA quality guides purifying selection.

Felix Thoma1,2, Johannes Hagen1,2, Romina Rathberger1,2

  • 1Faculty of Biology, Ludwig-Maximilians-Universität München, Planegg-Martinsried, Germany.

Plos Genetics
|January 9, 2026
PubMed
Summary
This summary is machine-generated.

Yeast cells actively maintain mitochondrial genome (mtDNA) quality by selecting against mutations that impair respiratory function. This process, known as mtDNA quality control, ensures cellular energy supply and prevents mitochondrial diseases.

More Related Videos

Genotyping Single Nucleotide Polymorphisms in the Mitochondrial Genome by Pyrosequencing
07:24

Genotyping Single Nucleotide Polymorphisms in the Mitochondrial Genome by Pyrosequencing

Published on: February 10, 2023

1.9K
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

4.3K

Related Experiment Videos

Last Updated: Jan 13, 2026

Author Spotlight: High-Throughput Image-Based Quantification of Mitochondrial DNA Synthesis and Distribution
10:47

Author Spotlight: High-Throughput Image-Based Quantification of Mitochondrial DNA Synthesis and Distribution

Published on: May 5, 2023

4.4K
Genotyping Single Nucleotide Polymorphisms in the Mitochondrial Genome by Pyrosequencing
07:24

Genotyping Single Nucleotide Polymorphisms in the Mitochondrial Genome by Pyrosequencing

Published on: February 10, 2023

1.9K
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

4.3K

Area of Science:

  • Cell Biology
  • Genetics
  • Biochemistry

Background:

  • The mitochondrial genome (mtDNA) is crucial for cellular respiration and energy production.
  • Mutations in mtDNA can lead to mitochondrial diseases due to impaired oxidative phosphorylation.
  • Mitochondrial DNA quality control (mtDNA-QC) is essential for maintaining cellular function.

Purpose of the Study:

  • To develop a high-throughput assay for tracking mtDNA segregation in yeast.
  • To investigate the mechanisms of mtDNA quality control under different conditions.
  • To understand how cells select for functional mitochondrial genomes.

Main Methods:

  • Developed a flow cytometry assay in Saccharomyces cerevisiae to track fluorescently labeled wild-type (WT) mtDNA versus unlabeled mutant mtDNA.
  • Analyzed mtDNA segregation in heteroplasmic zygotes.
  • Integrated cytometric data with growth assays, qPCR for mtDNA copy number, and simulations.
  • Used fluorescent reporters to assess local ATP levels and membrane potential.

Main Results:

  • Observed purifying selection against mtDNA mutations affecting complexes III, IV, and ATP synthase, even under non-respiratory conditions.
  • Decline of mtDNAΔatp6 was explained by reduced copy number and cellular proliferative disadvantage.
  • Loss of mtDNAΔcob and mtDNAΔcox2 indicated additional intracellular selection beyond growth defects.
  • Disruption of respiratory chain components abolished physiological gradients and impaired mtDNA-QC.

Conclusions:

  • Yeast cells employ intracellular selection mechanisms to maintain mtDNA integrity.
  • Local bioenergetic differences near mtDNA influence selection, acting as a proxy for genome function.
  • This process is vital for preventing mitochondrial dysfunction and disease.