ArticleMolecular cell2018
Topoisomerase 3α Is Required for Decatenation and Segregation of Human mtDNA.
Article in Molecular cell, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 81 papers.
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Who cites it
81 citing papers in PubMed, 130 citations in OpenAlex.
- The factory enters the fray: how mitochondrial protein trafficking shapes the host response to infection.Journal of virology · 2026Review
- R-loops and D-loops: a delicate balance in genomic stability and instability.Cell communication and signaling : CCS · 2026Review
- TOP1 and TOP2 complementarily maintain DNA replication fork progression in vertebrates.The Journal of biological chemistry · 2026Article
- The segregation of organelles and organellar genomes across eukaryotic biology.Biochemical Society transactions · 2026Review
- Epigenome-Wide Association Studies of Proteasome Inhibitor-Related Cardiotoxicity in Patients with Multiple Myeloma.Cancers · 2026Article
- Differential roles of type I topoisomerases in regulating HPV pathogenesis.Proceedings of the National Academy of Sciences of the United States of America · 2026Article
- Identification of aFrontiers in pharmacology · 2026Article
- Anellovirus protein encoded byProceedings of the National Academy of Sciences of the United States of America · 2025Article
- Proteolytic cleavage activates the mitochondrial isoform of TOP3A.Nucleic acids research · 2025Article
- Mitochondrial DNA Replication and Disease: A Historical Perspective on Molecular Insights and Therapeutic Advances.International journal of molecular sciences · 2025Review
- Topoisomerase IIIα controls alternative lengthening of telomeres.Cell reports · 2025Article
- MRE11-independent effects of Mirin on mitochondrial DNA integrity and cellular immune responses.Molecular biology of the cell · 2025Article
- Mechanisms and pathologies of human mitochondrial DNA replication and deletion formation.The Biochemical journal · 2024Review
- Linear DNA-driven recombination in mammalian mitochondria.Nucleic acids research · 2024Article
- Variation of Structure and Cellular Functions of Type IA Topoisomerases across the Tree of Life.Cells · 2024Review
- Single-nucleoid architecture reveals heterogeneous packaging of mitochondrial DNA.Nature structural & molecular biology · 2024Article
- A system for inducible mitochondria-specific protein degradation in vivo.Nature communications · 2024Article
- Mitochondrial DNA replication stress triggers a pro-inflammatory endosomal pathway of nucleoid disposal.Nature cell biology · 2024Article
- Inhibition of mitochondrial calcium transporters alters adp-induced platelet responses.Molecular biology reports · 2024Article
- TOP3A coupling with replication forks and repair of TOP3A cleavage complexes.Cell cycle (Georgetown, Tex.) · 2024Review
21 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
15 authors at 6 institutions in 4 countries.
Funding
Abstract
How mtDNA replication is terminated and the newly formed genomes are separated remain unknown. We here demonstrate that the mitochondrial isoform of topoisomerase 3α (Top3α) fulfills this function, acting independently of its nuclear role as a component of the Holliday junction-resolving BLM-Top3α-RMI1-RMI2 (BTR) complex. Our data indicate that mtDNA replication termination occurs via a hemicatenane formed at the origin of H-strand replication and that Top3α is essential for resolving this structure. Decatenation is a prerequisite for separation of the segregating unit of mtDNA, the nucleoid, within the mitochondrial network. The importance of this process is highlighted in a patient with mitochondrial disease caused by biallelic pathogenic variants in TOP3A, characterized by muscle-restricted mtDNA deletions and chronic progressive external ophthalmoplegia (CPEO) plus syndrome. Our work establishes Top3α as an essential component of the mtDNA replication machinery and as the first component of the mtDNA separation machinery.
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Registered trials
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