Related Experiment Video
Updated: Oct 26, 2026

High-Throughput Image-Based Quantification of Mitochondrial DNA Synthesis and Distribution
Published on: May 5, 2023
Mitochondrial DNA replication and genome stability: From mechanisms to therapeutic strategies
Maria Falkenberg1, Claes M Gustafsson1
1Department of Medical Biochemistry and Cell Biology, Institute of Biomedicine, University of Gothenburg, Gothenburg, Sweden.
Abstract:
Replication of the mitochondrial genome is essential to sustain cellular energy conversion, and defects in this process cause severe, progressive mitochondrial diseases that have long lacked curative treatment. This is now beginning to change: mechanistic insight into the replication machinery has started to translate into therapy, with the first disease-modifying treatment recently approved and others entering clinical evaluation. Here we review the molecular mechanisms of mitochondrial DNA replication, with emphasis on replication initiation, deletion formation, and replication completion, and discuss how insights into these processes are opening new therapeutic possibilities for treating mitochondrial disease.
Related Concept Videos
Replication in Eukaryotes
Many Proteins Orchestrate Replication at the Origin
Eukaryotic replication follows many of the same...
Replication in Eukaryotes
The DNA Replication Fork
Animal Mitochondrial Genetics
DNA Damage can Stall the Cell Cycle
DNA Damage Can Stall the Cell Cycle
