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
Covalently Linked Protein Regulators02:04

Covalently Linked Protein Regulators

8.7K
Proteins can undergo many types of post-translational modifications, often in response to changes in their environment. These modifications play an important role in the function and stability of these proteins. Covalently linked molecules include functional groups, such as methyl, acetyl, and phosphate groups, and also small proteins, such as ubiquitin. There are around 200 different types of covalent regulators that have been identified.
These groups modify specific amino acids in a protein....
8.7K
Comparing Mitochondrial, Chloroplast, and Prokaryotic Genomes02:16

Comparing Mitochondrial, Chloroplast, and Prokaryotic Genomes

15.1K
The present-day mitochondrial and chloroplast genomes have retained some of the characteristics of their ancestral prokaryotes and also have acquired new attributes during their evolution within eukaryotic cells. Like prokaryotic genomes, mitochondrial and chloroplast genomes neither bind with histone-like proteins nor show complex packaging into chromosome-like structures, as observed in eukaryotes. Unlike mitotic cell divisions observed in eukaryotic cells, mitochondria and chloroplasts...
15.1K
X-linked Traits01:19

X-linked Traits

58.3K
In most mammalian species, females have two X sex chromosomes and males have an X and Y. As a result, mutations on the X chromosome in females may be masked by the presence of a normal allele on the second X. In contrast, a mutation on the X chromosome in males more often causes observable biological defects, as there is no normal X to compensate. Trait variations arising from mutations on the X chromosome are called “X-linked”.
58.3K
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
Mechanistic Models: Overview of Compartment Models01:21

Mechanistic Models: Overview of Compartment Models

369
Mechanistic models, a category encompassing both physiological and compartmental modeling, differ from empirical models' approaches to incorporating known factors about the systems being modeled. Empirical models describe data with minimal assumptions, while mechanistic models aim to provide a robust description of available data by specifying assumptions and integrating known factors about the system. Compartmental analysis is a key example of a mechanistic model in pharmacokinetics and...
369

You might also read

Related Articles

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

Sort by
Same author

Analysis of Lesion Shrinkage and Factors Influencing Shrinkage Following Radiofrequency Ablation of Breast Nodules: An Observational Study.

Journal of investigative surgery : the official journal of the Academy of Surgical Research·2026
Same author

Dopamine-driven mitochondrial reverse electron transport in immune cells mediates gut-brain ROS signaling during sleep deprivation.

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

IRE1 regulates the proteostasis of TDP-43/TARDBP in ALS/FTD through ribosome-associated quality control.

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

EZH2 as regulator of stemness signature and driver of esophageal squamous cell carcinomas.

Scientific reports·2026
Same author

Spatiotemporal patterns of gene expression changes in the mouse dentate gyrus following entorhinal denervation.

Frontiers in molecular neuroscience·2026
Same author

Mitochondrial complex I as a master regulator of redox signaling: From structural architecture to directionality of electron transport.

Free radical biology & medicine·2026

Related Experiment Video

Updated: Jan 21, 2026

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

4.4K

MISTERMINATE Mechanistically Links Mitochondrial Dysfunction with Proteostasis Failure.

Zhihao Wu1, Ishaq Tantray1, Junghyun Lim2

  • 1Department of Pathology and Programs in Cancer Biology and Neurosciences, Stanford University School of Medicine, Stanford, CA, USA.

Molecular Cell
|August 6, 2019
PubMed
Summary

Mitochondrial dysfunction causes errors in protein production, leading to toxic protein buildup and cell death. This study reveals a new mechanism linking these issues, offering potential therapeutic targets for neurodegenerative diseases.

Keywords:
CAT-tailingMISTERMINATEPINK1/ParkinParkinson’s diseaseRQCmitochondrial stressneurodegenerationproteostasisribosome stallingtranslation termination

More Related Videos

A Rat Model of Pressure Overload Induced Moderate Remodeling and Systolic Dysfunction as Opposed to Overt Systolic Heart Failure
07:13

A Rat Model of Pressure Overload Induced Moderate Remodeling and Systolic Dysfunction as Opposed to Overt Systolic Heart Failure

Published on: April 30, 2020

6.9K
Author Spotlight: Investigating HR-Dependent Cardiac Function in Mouse Models Through a Novel Atrial-Pacing Approach
07:49

Author Spotlight: Investigating HR-Dependent Cardiac Function in Mouse Models Through a Novel Atrial-Pacing Approach

Published on: July 21, 2023

1.9K

Related Experiment Videos

Last Updated: Jan 21, 2026

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

4.4K
A Rat Model of Pressure Overload Induced Moderate Remodeling and Systolic Dysfunction as Opposed to Overt Systolic Heart Failure
07:13

A Rat Model of Pressure Overload Induced Moderate Remodeling and Systolic Dysfunction as Opposed to Overt Systolic Heart Failure

Published on: April 30, 2020

6.9K
Author Spotlight: Investigating HR-Dependent Cardiac Function in Mouse Models Through a Novel Atrial-Pacing Approach
07:49

Author Spotlight: Investigating HR-Dependent Cardiac Function in Mouse Models Through a Novel Atrial-Pacing Approach

Published on: July 21, 2023

1.9K

Area of Science:

  • Cell Biology
  • Neuroscience
  • Biochemistry

Background:

  • Mitochondrial dysfunction and proteostasis failure are key features of neurodegenerative diseases.
  • The precise relationship between these two pathologies remains unclear.

Purpose of the Study:

  • To investigate the mechanistic link between mitochondrial dysfunction and proteostasis failure.
  • To describe a novel phenomenon, MISTERMINATE, connecting these cellular defects.

Main Methods:

  • Utilized Drosophila and mammalian cell models.
  • Investigated the impact of mitochondrial dysfunction on translation termination of nuclear-encoded mitochondrial mRNAs.
  • Analyzed the consequences of C-terminally extended proteins on cellular health and disease models.

Main Results:

  • Mitochondrial dysfunction impairs translational termination, causing C-terminal extension of proteins like Complex-I 30kD subunit (C-I30).
  • C-terminally extended C-I30 is toxic, forms aggregates, and contributes to cellular degeneration.
  • Enhancing co-translational quality control mitigates C-I30 extension and rescues disease phenotypes in a Parkinson's disease model.

Conclusions:

  • MISTERMINATE mechanistically links mitochondrial dysfunction with proteostasis failure.
  • Efficient translation termination is crucial for maintaining mitochondrial health and proteome homeostasis.
  • This discovery opens new avenues for therapeutic interventions in neurodegenerative diseases.