Safeguarding mitochondrial genomes in higher eukaryotes

Comprehensive molecular characterization of mitochondrial genomes in human cancers

(2020) Yuan Yuan et al.   NATURE GENETICS

VDAC oligomers form mitochondrial pores to release mtDNA fragments and promote lupus-like disease

(2020) Jeonghan Kim et al.   SCIENCE

A bacterial cytidine deaminase toxin enables CRISPR-free mitochondrial base editing

(2020) Beverly Y. Mok et al.   NATURE

Copy-choice recombination during mitochondrial L-strand synthesis causes DNA deletions

(2019) Örjan Persson et al.   Nature Communications

Mechanisms of Mitochondrial DNA Deletion Formation

(2019) Nadee Nissanka et al.   TRENDS IN GENETICS

Error-Prone Replication through UV Lesions by DNA Polymerase θ Protects against Skin Cancers

(2019) Jung-Hoon Yoon et al.   CELL

Mitochondrial fragmentation drives selective removal of deleterious mtDNA in the germline

(2019) Toby Lieber et al.   NATURE

Germline selection shapes human mitochondrial DNA diversity

(2019) Wei Wei et al.   SCIENCE

The DNA Repair Nuclease MRE11A Functions as a Mitochondrial Protector and Prevents T Cell Pyroptosis and Tissue Inflammation

(2019) Yinyin Li et al.   Cell Metabolism

Mitohormesis, UPRmt, and the Complexity of Mitochondrial DNA Landscapes in Cancer

(2019) Timothy C. Kenny et al.   CANCER RESEARCH

A Brief History of Mitochondrial Pathologies

(2019) Salvatore DiMauro   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

DNA Polymerase θ Increases Mutational Rates in Mitochondrial DNA

(2018) Simon Wisnovsky et al.   ACS Chemical Biology

UPRmt regulation and output: a stress response mediated by mitochondrial-nuclear communication

(2018) Andrew Melber et al.   CELL RESEARCH

Mitochondrial DNA replication in mammalian cells: overview of the pathway

(2018) Maria Falkenberg   Essays in Biochemistry

Developmental Decline in the MicroRNA 199a (miR-199a)/miR-214 Cluster in Human Fetal Lung Promotes Type II Cell Differentiation by Upregulating Key Transcription Factors

(2018) Ritu Mishra et al.   MOLECULAR AND CELLULAR BIOLOGY

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

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

Segregation of mitochondrial DNA heteroplasmy through a developmental genetic bottleneck in human embryos

(2018) Vasileios I. Floros et al.   NATURE CELL BIOLOGY

Base-excision repair deficiency alone or combined with increased oxidative stress does not increase mtDNA point mutations in mice

(2018) Johanna H K Kauppila et al.   NUCLEIC ACIDS RESEARCH

BAK/BAX macropores facilitate mitochondrial herniation and mtDNA efflux during apoptosis

(2018) Kate McArthur et al.   SCIENCE

Linear mitochondrial DNA is rapidly degraded by components of the replication machinery

(2018) Viktoriya Peeva et al.   Nature Communications

The mitochondrial DNA polymerase gamma degrades linear DNA fragments precluding the formation of deletions

(2018) Nadee Nissanka et al.   Nature Communications

Mitochondrial inner membrane permeabilisation enables mtDNA release during apoptosis

(2018) Joel S Riley et al.   EMBO JOURNAL

Mitochondrial double-stranded RNA triggers antiviral signalling in humans

(2018) Ashish Dhir et al.   NATURE

Parkin and PINK1 mitigate STING-induced inflammation

(2018) Danielle A. Sliter et al.   NATURE

New mitochondrial DNA synthesis enables NLRP3 inflammasome activation

(2018) Zhenyu Zhong et al.   NATURE

MitoTALEN reduces mutant mtDNA load and restores tRNAAla levels in a mouse model of heteroplasmic mtDNA mutation

(2018) Sandra R. Bacman et al.   NATURE MEDICINE

Genome editing in mitochondria corrects a pathogenic mtDNA mutation in vivo

(2018) Payam A. Gammage et al.   NATURE MEDICINE

Single-Molecule Analysis of mtDNA Replication Uncovers the Basis of the Common Deletion

(2017) Aaron F. Phillips et al.   MOLECULAR CELL

Evidence for double-strand break mediated mitochondrial DNA replication in Saccharomyces cerevisiae

(2017) Kanchanjunga Prasai et al.   NUCLEIC ACIDS RESEARCH

Nucleotide pools dictate the identity and frequency of ribonucleotide incorporation in mitochondrial DNA

(2017) Anna-Karin Berglund et al.   PLoS Genetics

Incompatibility between Nuclear and Mitochondrial Genomes Contributes to an Interspecies Reproductive Barrier

(2016) Hong Ma et al.   Cell Metabolism

Microhomology-mediated end joining is the principal mediator of double-strand break repair during mitochondrial DNA lesions

(2016) Satish Kumar Tadi et al.   MOLECULAR BIOLOGY OF THE CELL

Mitochondrial and nuclear DNA matching shapes metabolism and healthy ageing

(2016) Ana Latorre-Pellicer et al.   NATURE

Maintenance and propagation of a deleterious mitochondrial genome by the mitochondrial unfolded protein response

(2016) Yi-Fan Lin et al.   NATURE

Accurate concentration control of mitochondria and nucleoids

(2016) R. Jajoo et al.   SCIENCE

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

(2016) Samantha C. Lewis et al.   SCIENCE

Mitochondrial DNA stress primes the antiviral innate immune response

(2015) A. Phillip West et al.   NATURE

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

Integrity of the yeast mitochondrial genome, but not its distribution and inheritance, relies on mitochondrial fission and fusion

(2015) Christof Osman et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The exonuclease activity of DNA polymerase γ is required for ligation during mitochondrial DNA replication

(2015) Bertil Macao et al.   Nature Communications

Selections that isolate recombinant mitochondrial genomes in animals

(2015) Hansong Ma et al.   eLife

Mitochondrially targeted ZFNs for selective degradation of pathogenic mitochondrial genomes bearing large-scale deletions or point mutations

(2014) P. A. Gammage et al.   EMBO Molecular Medicine

Linear mtDNA fragments and unusual mtDNA rearrangements associated with pathological deficiency of MGME1 exonuclease

(2014) Thomas J. Nicholls et al.   HUMAN MOLECULAR GENETICS

Oxidative Stress Is Not a Major Contributor to Somatic Mitochondrial DNA Mutations

(2014) Leslie S. Itsara et al.   PLoS Genetics

Origins and functional consequences of somatic mitochondrial DNA mutations in human cancer

(2014) Young Seok Ju et al.   eLife

To be or not to be a nucleoid protein: A comparison of mass-spectrometry based approaches in the identification of potential mtDNA-nucleoid associated proteins

(2013) Fenna Hensen et al.   BIOCHIMIE

Specific elimination of mutant mitochondrial genomes in patient-derived cells by mitoTALENs

(2013) Sandra R Bacman et al.   NATURE MEDICINE

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

(2013) E. Hagstrom 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

Dynamics of nucleoid structure regulated by mitochondrial fission contributes to cristae reformation and release of cytochrome c

(2013) R. Ban-Ishihara et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Ultra-Sensitive Sequencing Reveals an Age-Related Increase in Somatic Mitochondrial Mutations That Are Inconsistent with Oxidative Damage

(2013) Scott R. Kennedy et al.   PLoS Genetics

Minimizing the damage: repair pathways keep mitochondrial DNA intact

(2012) Lawrence Kazak et al.   NATURE REVIEWS MOLECULAR CELL 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

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

Discovery of a Novel Function for Human Rad51

(2010) Jay M. Sage et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A role for mitochondria in NLRP3 inflammasome activation

(2010) Rongbin Zhou et al.   NATURE

Mitochondrial DNA mutations and human disease

(2009) Helen A.L. Tuppen et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Novel DNA mismatch-repair activity involving YB-1 in human mitochondria

(2009) Nadja C. de Souza-Pinto et al.   DNA REPAIR

Mitochondrial Localization of PARP-1 Requires Interaction with Mitofilin and Is Involved in the Maintenance of Mitochondrial DNA Integrity

(2009) Marianna N. Rossi et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Intra- and inter-molecular recombination of mitochondrial DNA after in vivo induction of multiple double-strand breaks

(2009) S. R. Bacman et al.   NUCLEIC ACIDS RESEARCH

Oxidative stress induces degradation of mitochondrial DNA

(2009) Inna Shokolenko et al.   NUCLEIC ACIDS RESEARCH

Anticancer DNA intercalators cause p53-dependent mitochondrial DNA nucleoid re-modelling

(2009) N Ashley et al.   ONCOGENE

Mechanisms of formation and accumulation of mitochondrial DNA deletions in aging neurons

(2008) Hirokazu Fukui et al.   HUMAN MOLECULAR GENETICS

Development of a single-chain, quasi-dimeric zinc-finger nuclease for the selective degradation of mutated human mitochondrial DNA

(2008) Michal Minczuk et al.   NUCLEIC ACIDS RESEARCH

Manipulating the Metazoan Mitochondrial Genome with Targeted Restriction Enzymes

(2008) H. Xu et al.   SCIENCE

A Mouse Model of Mitochondrial Disease Reveals Germline Selection Against Severe mtDNA Mutations

(2008) Weiwei Fan et al.   SCIENCE