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

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...

You might also read

Related Articles

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

Sort by

Same author

Self-splicing RNA circularization facilitated by intact group I and II introns.

Nature communications·2025

Same author

Huaier suppresses lung cancer by simultaneously and independently inhibiting the antioxidant pathway SLC7A11/GPX4 while enhancing ferritinophagy.

Cell death discovery·2025

Same author

Prime editor-based high-throughput screening reveals functional synonymous mutations in human cells.

Nature biotechnology·2025

Same author

UNC0638 inhibits SARS-CoV-2 entry by blocking cathepsin L maturation.

Journal of virology·2025

Same author

A mitochondrial disease model is generated and corrected using engineered base editors in rat zygotes.

Nature biotechnology·2025

Same author

Efficient mitochondrial A-to-G base editors for the generation of mitochondrial disease models.

Nature biotechnology·2025

Same journal

Family of magnetic field-boosted superconductors in rhombohedral graphene.

Nature·2026

Same journal

What's the human cost of US research turmoil? A new film finds out.

Nature·2026

Same journal

Daily briefing: Ovaries start a second job after menopause.

Nature·2026

Same journal

Audio long read: Is the peptide craze backed by science? The promise behind the hype.

Nature·2026

Same journal

Scientists fight back against far-right plans to restrict academic freedom in Germany.

Nature·2026

Same journal

How AI can crack open the 'hidden curriculum' for neurodivergent students.

Nature·2026

See all related articles

Search research articles

Related Experiment Video

Updated: May 31, 2025

Generation of Human Brain Organoids for Mitochondrial Disease Modeling

Generation of Human Brain Organoids for Mitochondrial Disease Modeling

Published on: June 21, 2021

Precise modelling of mitochondrial diseases using optimized mitoBEs.

Xiaoxue Zhang^1,2, Xue Zhang¹, Jiwu Ren^1,3

¹Changping Laboratory, Beijing, The People's Republic of China.

|January 22, 2025

Summary

This summary is machine-generated.

Researchers developed improved mitochondrial base editors (mitoBEs v2) to create accurate animal models for mitochondrial diseases. These base editors efficiently correct mitochondrial DNA mutations with no detectable off-target effects, paving the way for new disease therapies.

More Related Videos

An In Vitro Approach to Study Mitochondrial Dysfunction: A Cybrid Model

An In Vitro Approach to Study Mitochondrial Dysfunction: A Cybrid Model

Published on: March 9, 2022

Author Spotlight: An Optimized Automated Method for Investigating Retinoic Acid Receptors in Neuronal Mitochondria

Author Spotlight: An Optimized Automated Method for Investigating Retinoic Acid Receptors in Neuronal Mitochondria

Published on: July 28, 2023

Related Experiment Videos

Last Updated: May 31, 2025

Generation of Human Brain Organoids for Mitochondrial Disease Modeling

Generation of Human Brain Organoids for Mitochondrial Disease Modeling

Published on: June 21, 2021

An In Vitro Approach to Study Mitochondrial Dysfunction: A Cybrid Model

An In Vitro Approach to Study Mitochondrial Dysfunction: A Cybrid Model

Published on: March 9, 2022

Author Spotlight: An Optimized Automated Method for Investigating Retinoic Acid Receptors in Neuronal Mitochondria

Author Spotlight: An Optimized Automated Method for Investigating Retinoic Acid Receptors in Neuronal Mitochondria

Published on: July 28, 2023

Area of Science:

Genetics
Molecular Biology
Mitochondrial Biology

Background:

Mitochondrial diseases are a group of debilitating genetic disorders.
Accurate animal models are essential for understanding and treating these diseases.
Existing base editing tools have limitations in targeting mitochondrial DNA.

Purpose of the Study:

To develop and optimize mitochondrial base editors (mitoBEs) for enhanced accuracy and efficiency.
To establish a foundation for creating novel mouse models of mitochondrial diseases.
To investigate the therapeutic potential of base editing in mitochondrial DNA.

Main Methods:

Optimized adenine and cytosine deaminases to create mitoBEs v2.
Utilized circular RNA-encoded mitoBEs v2 to target 70 mouse mitochondrial DNA mutations.
Developed single-base-editing mouse models by optimizing transcription activator-like effector (TALE) binding sites.

Main Results:

Achieved up to 82% editing efficiency in mice with no detectable nuclear off-target effects.
Demonstrated persistence and maternal inheritance of edited mitochondrial DNA across generations.
Successfully created mouse models for mt-Nd5 A12784G and mt-Atp6 T8591C mutations, recapitulating disease phenotypes.

Conclusions:

Upgraded mitoBEs v2 provide a highly efficient and precise strategy for constructing mitochondrial disease models.
These models enable the study of disease mechanisms and the evaluation of therapeutic interventions.
The developed mitoBEs hold significant promise for advancing research and treatment of mitochondrial disorders.