Controlling the topology of mammalian mitochondrial DNA

High-resolution imaging reveals compartmentalization of mitochondrial protein synthesis in cultured human cells

(2021) Matthew Zorkau et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mechanism of membrane-tethered mitochondrial protein synthesis

(2021) Yuzuru Itoh et al.   SCIENCE

Predominant cellular mitochondrial dysfunction in the TOP3A gene-caused Bloom syndrome-like disorder

(2021) Wenjun Jiang et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

The mitochondrial single-stranded DNA binding protein is essential for initiation of mtDNA replication

(2021) Min Jiang et al.   Science Advances

The many lives of type IA topoisomerases

(2020) Anna H. Bizard et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

ER-mitochondria contacts promote mtDNA nucleoids active transportation via mitochondrial dynamic tubulation

(2020) Jinshan Qin et al.   Nature Communications

Topoisomerase III Acts at the Replication Fork to Remove Precatenanes

(2019) Chong M. Lee et al.   JOURNAL OF BACTERIOLOGY

The mitochondrial type IB topoisomerase drives mitochondrial translation and carcinogenesis

(2019) S. A. Baechler et al.   Nature Communications

RNase H1 directs origin-specific initiation of DNA replication in human mitochondria

(2019) Viktor Posse et al.   PLoS Genetics

TEFM regulates both transcription elongation and RNA processing in mitochondria

(2019) Shan Jiang et al.   EMBO REPORTS

Twist and Turn—Topoisomerase Functions in Mitochondrial DNA Maintenance

(2019) Steffi Goffart et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

PICH and TOP3A cooperate to induce positive DNA supercoiling

(2019) Anna H. Bizard et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Beyond the unwinding: Role of TOP1MT in mitochondrial translation

(2019) Simone A Baechler et al.   CELL CYCLE

Organization of DNA in Mammalian Mitochondria

(2019) Géraldine Farge et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Functional Interplay between Cristae Biogenesis, Mitochondrial Dynamics and Mitochondrial DNA Integrity

(2019) Arun Kumar Kondadi et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Mitochondrial DNA replication in mammalian cells: overview of the pathway

(2018) Maria Falkenberg   Essays in Biochemistry

ATAD3 controls mitochondrial cristae structure in mouse muscle, influencing mtDNA replication and cholesterol levels

(2018) Susana Peralta et al.   JOURNAL OF CELL SCIENCE

Topoisomerase 3α Is Required for Decatenation and Segregation of Human mtDNA

(2018) Thomas J. Nicholls et al.   MOLECULAR CELL

Mice lacking the mitochondrial exonuclease MGME1 accumulate mtDNA deletions without developing progeria

(2018) Stanka Matic et al.   Nature Communications

Mutations in TOP3A Cause a Bloom Syndrome-like Disorder

(2018) Carol-Anne Martin et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Failure is not an option – mitochondrial genome segregation in trypanosomes

(2018) André Schneider et al.   JOURNAL OF CELL SCIENCE

Ciprofloxacin impairs mitochondrial DNA replication initiation through inhibition of Topoisomerase 2

(2018) Anu Hangas et al.   NUCLEIC ACIDS RESEARCH

Separating and Segregating the Human Mitochondrial Genome

(2018) Thomas J. Nicholls et al.   TRENDS IN BIOCHEMICAL SCIENCES

MitoMiner v4.0: an updated database of mitochondrial localization evidence, phenotypes and diseases

(2018) Anthony C Smith et al.   NUCLEIC ACIDS RESEARCH

Anthracycline cardiotoxicity: an update on mechanisms, monitoring and prevention

(2017) Peter A Henriksen   HEART

Transcription profiling suggests that mitochondrial topoisomerase IB acts as a topological barrier and regulator of mitochondrial DNA transcription

(2017) Ilaria Dalla Rosa et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

MtDNA-maintenance defects: syndromes and genes

(2017) Carlo Viscomi et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Regulation of Mammalian Mitochondrial Gene Expression: Recent Advances

(2017) Sarah F. Pearce et al.   TRENDS IN BIOCHEMICAL SCIENCES

Distribution bias and biochemical characterization of TOP1MT single nucleotide variants

(2017) Hongliang Zhang et al.   Scientific Reports

Unexpected sequences and structures of mtDNA required for efficient transcription from the first heavy-strand promoter

(2017) Akira Uchida et al.   eLife

Maintenance and Expression of Mammalian Mitochondrial DNA

(2016) Claes M. Gustafsson et al.   Annual Review of Biochemistry

RNA Polymerase II Regulates Topoisomerase 1 Activity to Favor Efficient Transcription

(2016) Laura Baranello et al.   CELL

Mitochondrial Protein Synthesis Adapts to Influx of Nuclear-Encoded Protein

(2016) Ricarda Richter-Dennerlein et al.   CELL

Human Mitochondrial Transcription Initiation Complexes Have Similar Topology on the Light and Heavy Strand Promoters

(2016) Yaroslav I. Morozov et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Human Mitochondrial Transcription Factor B2 Is Required for Promoter Melting during Initiation of Transcription

(2016) Viktor Posse et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mitochondrial RNA granules: Compartmentalizing mitochondrial gene expression

(2016) Alexis A. Jourdain et al.   JOURNAL OF CELL BIOLOGY

Roles of eukaryotic topoisomerases in transcription, replication and genomic stability

(2016) Yves Pommier et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Mitochondrial transcription termination factor 1 directs polar replication fork pausing

(2016) Yonghong Shi et al.   NUCLEIC ACIDS RESEARCH

Pathological ribonuclease H1 causes R-loop depletion and aberrant DNA segregation in mitochondria

(2016) Gokhan Akman et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

DNA and RNA topoisomerase activities of Top3β are promoted by mediator protein Tudor domain-containing protein 3

(2016) Grace Ee-Lu Siaw et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

ER-mitochondria contacts couple mtDNA synthesis with mitochondrial division in human cells

(2016) Samantha C. Lewis et al.   SCIENCE

Mitochondrial diseases

(2016) Gráinne S. Gorman et al.   Nature Reviews Disease Primers

TEFM is a potent stimulator of mitochondrial transcription elongation in vitro

(2015) Viktor Posse et al.   NUCLEIC ACIDS RESEARCH

Regulation of DNA replication at the end of the mitochondrial D-loop involves the helicase TWINKLE and a conserved sequence element

(2015) Elisabeth Jemt et al.   NUCLEIC ACIDS RESEARCH

Cross-strand binding of TFAM to a single mtDNA molecule forms the mitochondrial nucleoid

(2015) Christian Kukat et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

PICH promotes sister chromatid disjunction and co-operates with topoisomerase II in mitosis

(2015) Christian F. Nielsen et al.   Nature Communications

Human Mitochondrial DNA-Protein Complexes Attach to a Cholesterol-Rich Membrane Structure

(2015) Joachim M. Gerhold et al.   Scientific Reports

Whole Cell Formaldehyde Cross-Linking Simplifies Purification of Mitochondrial Nucleoids and Associated Proteins Involved in Mitochondrial Gene Expression

(2015) Nina Rajala et al.   PLoS One

In D-loop: 40years of mitochondrial 7S DNA

(2014) Thomas J. Nicholls et al.   EXPERIMENTAL GERONTOLOGY

Mapping Topoisomerase Sites in Mitochondrial DNA with a Poisonous Mitochondrial Topoisomerase I (Top1mt)

(2014) Ilaria Dalla Rosa et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Arginine Methylation Facilitates the Recruitment of TOP3B to Chromatin to Prevent R Loop Accumulation

(2014) Yanzhong Yang et al.   MOLECULAR CELL

Structural and mechanistic insight into Holliday-junction dissolution by Topoisomerase IIIα and RMI1

(2014) Nicolas Bocquet et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Increased negative supercoiling of mtDNA in TOP1mt knockout mice and presence of topoisomerases II  and II  in vertebrate mitochondria

(2014) H. Zhang et al.   NUCLEIC ACIDS RESEARCH

Distinct structural features of TFAM drive mitochondrial DNA packaging versus transcriptional activation

(2014) Huu B. Ngo et al.   Nature Communications

In Vitro-Reconstituted Nucleoids Can Block Mitochondrial DNA Replication and Transcription

(2014) Géraldine Farge et al.   Cell Reports

MTERF1 Binds mtDNA to Prevent Transcriptional Interference at the Light-Strand Promoter but Is Dispensable for rRNA Gene Transcription Regulation

(2013) Mügen Terzioglu et al.   Cell Metabolism

Tendon Injury and Fluoroquinolone Use: A Systematic Review

(2013) Anne L. Stephenson et al.   DRUG SAFETY

Topoisomerases facilitate transcription of long genes linked to autism

(2013) Ian F. King et al.   NATURE

Loss-of-function mutations in MGME1 impair mtDNA replication and cause multisystemic mitochondrial disease

(2013) Cornelia Kornblum et al.   NATURE GENETICS

Top3β is an RNA topoisomerase that works with fragile X syndrome protein to promote synapse formation

(2013) Dongyi Xu et al.   NATURE NEUROSCIENCE

Deletion of TOP3β, a component of FMRP-containing mRNPs, contributes to neurodevelopmental disorders

(2013) Georg Stoll et al.   NATURE NEUROSCIENCE

Negative regulation of mitochondrial transcription by mitochondrial topoisomerase I

(2013) Stefan Sobek et al.   NUCLEIC ACIDS RESEARCH

No recombination of mtDNA after heteroplasmy for 50 generations in the mouse maternal germline

(2013) E. Hagstrom et al.   NUCLEIC ACIDS RESEARCH

Mitochondrial DNA replication proceeds via a ‘bootlace’ mechanism involving the incorporation of processed transcripts

(2013) Aurelio Reyes et al.   NUCLEIC ACIDS RESEARCH

Replication factors transiently associate with mtDNA at the mitochondrial inner membrane to facilitate replication

(2013) N. Rajala et al.   NUCLEIC ACIDS RESEARCH

Genome-Wide Analysis Reveals Coating of the Mitochondrial Genome by TFAM

(2013) Yun E. Wang et al.   PLoS One

Gene regulation and priming by topoisomerase IIα in embryonic stem cells

(2013) Sudhir Thakurela et al.   Nature Communications

ER-associated mitochondrial division links the distribution of mitochondria and mitochondrial DNA in yeast

(2013) Andrew Murley et al.   eLife

RECQL4 localizes to mitochondria and preserves mitochondrial DNA integrity

(2012) Deborah L. Croteau et al.   AGING CELL

RecQL4 cytoplasmic localization: Implications in mitochondrial DNA oxidative damage repair

(2012) Zhenfen Chi et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Preventing Replication Stress to Maintain Genome Stability: Resolving Conflicts between Replication and Transcription

(2012) Rodrigo Bermejo et al.   MOLECULAR CELL

Decatenation of DNA by the S. cerevisiae Sgs1-Top3-Rmi1 and RPA Complex: A Mechanism for Disentangling Chromosomes

(2012) Petr Cejka et al.   MOLECULAR CELL

Mitochondrial nucleoid interacting proteins support mitochondrial protein synthesis

(2012) J. He et al.   NUCLEIC ACIDS RESEARCH

A hybrid G-quadruplex structure formed between RNA and DNA explains the extraordinary stability of the mitochondrial R-loop

(2012) P. H. Wanrooij et al.   NUCLEIC ACIDS RESEARCH

Mitochondrial Topoisomerase I is Critical for Mitochondrial Integrity and Cellular Energy Metabolism

(2012) Céline Douarre et al.   PLoS One

Correlative 3D superresolution fluorescence and electron microscopy reveal the relationship of mitochondrial nucleoids to membranes

(2012) B. G. Kopek et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mammalian transcription factor A is a core component of the mitochondrial transcription machinery

(2012) Y. Shi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Target genes of Topoisomerase II  regulate neuronal survival and are defined by their chromatin state

(2012) V. K. Tiwari et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Human Mitochondrial DNA Replication

(2012) I. J. Holt et al.   Cold Spring Harbor Perspectives in Biology

Protein sliding and DNA denaturation are essential for DNA organization by human mitochondrial transcription factor A

(2012) Géraldine Farge et al.   Nature Communications

RNA processing in human mitochondria

(2011) Maria I.G. Lopez Sanchez et al.   CELL CYCLE

Superresolution Fluorescence Imaging of Mitochondrial Nucleoids Reveals Their Spatial Range, Limits, and Membrane Interaction

(2011) T. A. Brown et al.   MOLECULAR AND CELLULAR BIOLOGY

All tangled up: how cells direct, manage and exploit topoisomerase function

(2011) Seychelle M. Vos et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Human mitochondrial transcription factor A induces a U-turn structure in the light strand promoter

(2011) Anna Rubio-Cosials et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

The mitochondrial transcription and packaging factor Tfam imposes a U-turn on mitochondrial DNA

(2011) Huu B Ngo et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Transcriptional activation by mitochondrial transcription factor A involves preferential distortion of promoter DNA

(2011) Christopher S. Malarkey et al.   NUCLEIC ACIDS RESEARCH

TEFM (c17orf42) is necessary for transcription of human mtDNA

(2011) Michal Minczuk et al.   NUCLEIC ACIDS RESEARCH

Super-resolution microscopy reveals that mammalian mitochondrial nucleoids have a uniform size and frequently contain a single copy of mtDNA

(2011) C. Kukat et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Involvement of human ELAC2 gene product in 3' end processing of mitochondrial tRNAs

(2011) Lien K. Brzezniak et al.   RNA Biology

Positive Supercoiling of Mitotic DNA Drives Decatenation by Topoisomerase II in Eukaryotes

(2011) J. Baxter et al.   SCIENCE

The Mitochondrial Proteome and Human Disease

(2010) Sarah E. Calvo et al.   Annual Review of Genomics and Human Genetics

Human Topoisomerase IIIα Is a Single-stranded DNA Decatenase That Is Stimulated by BLM and RMI1

(2010) Jay Yang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mammalian Mitochondrial DNA Replication Intermediates Are Essentially Duplex but Contain Extensive Tracts of RNA/DNA Hybrid

(2010) Jaakko L.O. Pohjoismäki et al.   JOURNAL OF MOLECULAR BIOLOGY

Overexpression of MTERFD1 or MTERFD3 impairs the completion of mitochondrial DNA replication

(2010) Anne K. Hyvärinen et al.   MOLECULAR BIOLOGY REPORTS

Mitochondrial RNA Polymerase Is Needed for Activation of the Origin of Light-Strand DNA Replication

(2010) Javier Miralles Fusté et al.   MOLECULAR CELL

Rmi1 stimulates decatenation of double Holliday junctions during dissolution by Sgs1–Top3

(2010) Petr Cejka et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Maintenance of respiratory chain function in mouse hearts with severely impaired mtDNA transcription

(2010) Christoph Freyer et al.   NUCLEIC ACIDS RESEARCH

G-quadruplex structures in RNA stimulate mitochondrial transcription termination and primer formation

(2010) P. H. Wanrooij et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

TFB2 Is a Transient Component of the Catalytic Site of the Human Mitochondrial RNA Polymerase

(2009) Marina Sologub et al.   CELL

Human Heart Mitochondrial DNA Is Organized in Complex Catenated Networks Containing Abundant Four-way Junctions and Replication Forks

(2009) Jaakko L. O. Pohjoismäki et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Adaptation of topoisomerase I paralogs to nuclear and mitochondrial DNA

(2009) Ilaria Dalla Rosa et al.   NUCLEIC ACIDS RESEARCH

Phylogenomics of DNA topoisomerases: their origin and putative roles in the emergence of modern organisms

(2009) P. Forterre et al.   NUCLEIC ACIDS RESEARCH

Mitochondrial Topoisomerase I Sites in the Regulatory D-Loop Region of Mitochondrial DNA†

(2008) Hongliang Zhang et al.   BIOCHEMISTRY

Resolution of Sister Centromeres Requires RanBP2-Mediated SUMOylation of Topoisomerase IIα

(2008) Meelad M. Dawlaty et al.   CELL

RNase P without RNA: Identification and Functional Reconstitution of the Human Mitochondrial tRNA Processing Enzyme

(2008) Johann Holzmann et al.   CELL

BLAP18/RMI2, a novel OB-fold-containing protein, is an essential component of the Bloom helicase-double Holliday junction dissolvasome

(2008) T. R. Singh et al.   GENES & DEVELOPMENT

RMI, a new OB-fold complex essential for Bloom syndrome protein to maintain genome stability

(2008) D. Xu et al.   GENES & DEVELOPMENT

Mitochondrial nucleoids maintain genetic autonomy but allow for functional complementation

(2008) Robert W. Gilkerson et al.   JOURNAL OF CELL BIOLOGY

Resolution of Converging Replication Forks by RecQ and Topoisomerase III

(2008) Catherine Suski et al.   MOLECULAR CELL

Topoisomerase II Inactivation Prevents the Completion of DNA Replication in Budding Yeast

(2008) Jonathan Baxter et al.   MOLECULAR CELL

The mitochondrial DNA genetic bottleneck results from replication of a subpopulation of genomes

(2008) Timothy Wai et al.   NATURE GENETICS

Human mitochondrial RNA polymerase primes lagging-strand DNA synthesis in vitro

(2008) S. Wanrooij et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA