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

Mouse Models of Cancer Study

Mouse Models of Cancer Study

Mice have long served as models for studying human biology and pathology because of their phylogenetic and physiological similarity with humans. They are also easy to maintain and breed in the laboratory, and hence, many inbred strains are now available for research. Studies on mice have contributed immeasurably to our understanding of cancer biology.
The development of transgenic, knockout, and knock-in mice has led to an exponential increase in their use as model organisms in research,...

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...

Transcription Factors

Transcription Factors

Tissue-specific transcription factors contribute to diverse cellular functions in mammals. For example, the gene for beta globin, a major component of hemoglobin, is present in all cells of the body. However, it is only expressed in red blood cells because the transcription factors that can bind to the promoter sequences of the beta globin gene are only expressed in these cells. Tissue-specific transcription factors also ensure that mutations in these factors may impair only the function of...

You might also read

Related Articles

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

Sort by

Same author

Dopaminergic neurons preferentially accumulate mtDNA rearrangements.

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

Same author

Persistence of large mtDNA rearrangements linked to premature aging in Pol γ exonuclease-deficient mice.

Nucleic acids research·2026

Same author

Generation and characterization of the TRE-TNFR2 transgenic mouse for cell-specific and timed (over)expression of tumor necrosis factor receptor 2.

Cytokine·2026

Same author

The Mitochondrial Blueprint of Skin Aging: From Damage Signals to Dermatologic Interventions.

Aging and disease·2026

Same author

Loss-of-function variants in the CAPN1 activator CD99L2 cause X-linked spastic ataxia.

Nature communications·2026

Same author

Transient muscle expression of mitoARCUS in mice leads to sustained reductions in pathogenic mtDNA and reduces fatigability.

Molecular therapy : the journal of the American Society of Gene Therapy·2025

Search research articles

Related Experiment Video

Updated: May 27, 2026

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

Mitochondrial transcription: lessons from mouse models.

Susana Peralta¹, Xiao Wang, Carlos T Moraes

¹University of Miami Miller School of Medicine, Department of Neurology, Miami, FL 33136, USA.

Biochimica Et Biophysica Acta

|November 29, 2011

Summary

Knockout mouse models have been crucial for understanding mammalian mitochondrial DNA (mtDNA) transcription. Further research is needed to identify unknown transcription factors and regulatory mechanisms.

More Related Videos

Single Cell Transcriptional Profiling of Adult Mouse Cardiomyocytes

Single Cell Transcriptional Profiling of Adult Mouse Cardiomyocytes

Published on: December 28, 2011

Isolation of Mitochondria from Mouse Skeletal Muscle for Respirometric Assays

Isolation of Mitochondria from Mouse Skeletal Muscle for Respirometric Assays

Published on: February 10, 2022

Related Experiment Videos

Last Updated: May 27, 2026

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

Single Cell Transcriptional Profiling of Adult Mouse Cardiomyocytes

Single Cell Transcriptional Profiling of Adult Mouse Cardiomyocytes

Published on: December 28, 2011

Isolation of Mitochondria from Mouse Skeletal Muscle for Respirometric Assays

Isolation of Mitochondria from Mouse Skeletal Muscle for Respirometric Assays

Published on: February 10, 2022

Area of Science:

Molecular Biology
Genetics
Biochemistry

Background:

Mammalian mitochondrial DNA (mtDNA) encodes key proteins for ATP production via oxidative phosphorylation (OXPHOS).
Nuclear DNA encodes the remaining OXPHOS components and mitochondrial translation machinery.
Regulation of mtDNA gene expression is vital for cellular energy production and disease processes.

Purpose of the Study:

To review the contribution of knockout mouse models to understanding mammalian mitochondrial transcription.
To highlight current knowledge gaps in mtDNA gene expression regulation.

Main Methods:

Review of in vitro and in vivo studies.
Analysis of phenotypes from knockout mouse strains.
Focus on contributions to understanding mitochondrial transcription in mammals.

Main Results:

Knockout mouse models have significantly advanced in vivo understanding of mtDNA transcription.
The core machinery for basal mtDNA transcription is partially identified.
Many transcription factors and regulatory mechanisms remain unknown.

Conclusions:

Mitochondrial transcription research heavily relies on insights from knockout mouse models.
Further investigation is required to elucidate the complete mtDNA transcription machinery and its regulation under various physiological and pathological conditions.