DNA replication and transcription in mammalian mitochondria.

The fidelity of DNA synthesis by eukaryotic replicative and translesion synthesis polymerases. Cell Res (2008) 3.15

NOTCH reprograms mitochondrial metabolism for proinflammatory macrophage activation. J Clin Invest (2015) 2.93

DNA methyltransferase 1, cytosine methylation, and cytosine hydroxymethylation in mammalian mitochondria. Proc Natl Acad Sci U S A (2011) 2.21

Mitochondrial translation and beyond: processes implicated in combined oxidative phosphorylation deficiencies. J Biomed Biotechnol (2010) 2.01

Oxidative stress, mitochondrial dysfunction, and aging. J Signal Transduct (2011) 1.94

Super-resolution microscopy reveals that mammalian mitochondrial nucleoids have a uniform size and frequently contain a single copy of mtDNA. Proc Natl Acad Sci U S A (2011) 1.90

The pathophysiology of mitochondrial disease as modeled in the mouse. Genes Dev (2009) 1.87

Defects in mitochondrial DNA replication and human disease. Crit Rev Biochem Mol Biol (2011) 1.86

Impaired mitochondrial transport and Parkin-independent degeneration of respiratory chain-deficient dopamine neurons in vivo. Proc Natl Acad Sci U S A (2011) 1.82

Expression and maintenance of mitochondrial DNA: new insights into human disease pathology. Am J Pathol (2008) 1.78

TFB2 is a transient component of the catalytic site of the human mitochondrial RNA polymerase. Cell (2009) 1.75

PrimPol, an archaic primase/polymerase operating in human cells. Mol Cell (2013) 1.72

The mitochondrial transcription and packaging factor Tfam imposes a U-turn on mitochondrial DNA. Nat Struct Mol Biol (2011) 1.68

Mitochondrial DNA mutations in disease and aging. J Cell Biol (2011) 1.67

Mitochondrial biology. Replication-transcription switch in human mitochondria. Science (2015) 1.64

Ultra-deep sequencing of mouse mitochondrial DNA: mutational patterns and their origins. PLoS Genet (2011) 1.60

Human mitochondrial mRNAs--like members of all families, similar but different. Biochim Biophys Acta (2010) 1.53

Human mitochondrial RNA polymerase primes lagging-strand DNA synthesis in vitro. Proc Natl Acad Sci U S A (2008) 1.52

Nuclear outsourcing of RNA interference components to human mitochondria. PLoS One (2011) 1.49

Origins and functional consequences of somatic mitochondrial DNA mutations in human cancer. Elife (2014) 1.39

Consequences of zygote injection and germline transfer of mutant human mitochondrial DNA in mice. Proc Natl Acad Sci U S A (2015) 1.39

TEFM (c17orf42) is necessary for transcription of human mtDNA. Nucleic Acids Res (2011) 1.38

Mammalian transcription factor A is a core component of the mitochondrial transcription machinery. Proc Natl Acad Sci U S A (2012) 1.37

Core human mitochondrial transcription apparatus is a regulated two-component system in vitro. Proc Natl Acad Sci U S A (2010) 1.34

Direct regulation of complex I by mitochondrial MEF2D is disrupted in a mouse model of Parkinson disease and in human patients. J Clin Invest (2011) 1.30

Mitochondrial DNA mutations and aging: devils in the details? Trends Genet (2008) 1.30

Phosphorylation of human TFAM in mitochondria impairs DNA binding and promotes degradation by the AAA+ Lon protease. Mol Cell (2012) 1.28

A compendium of human mitochondrial gene expression machinery with links to disease. Environ Mol Mutagen (2010) 1.26

Mitochondrial mechanisms of estrogen neuroprotection. Biochim Biophys Acta (2009) 1.25

Human mitochondrial RNA turnover caught in flagranti: involvement of hSuv3p helicase in RNA surveillance. Nucleic Acids Res (2009) 1.25

Interplay between innate immunity and negative-strand RNA viruses: towards a rational model. Microbiol Mol Biol Rev (2011) 1.22

ERAL1 is associated with mitochondrial ribosome and elimination of ERAL1 leads to mitochondrial dysfunction and growth retardation. Nucleic Acids Res (2010) 1.21

PDX1 deficiency causes mitochondrial dysfunction and defective insulin secretion through TFAM suppression. Cell Metab (2009) 1.21

Damaged mitochondrial DNA replication system and the development of diabetic retinopathy. Antioxid Redox Signal (2012) 1.21

Pentatricopeptide repeat domain protein 1 lowers the levels of mitochondrial leucine tRNAs in cells. Nucleic Acids Res (2009) 1.19

Mitochondrial genome deletions and minicircles are common in lice (Insecta: Phthiraptera). BMC Genomics (2011) 1.18

Apoptosis induced by persistent single-strand breaks in mitochondrial genome: critical role of EXOG (5'-EXO/endonuclease) in their repair. J Biol Chem (2011) 1.18

Physical and functional interactions between human mitochondrial single-stranded DNA-binding protein and tumour suppressor p53. Nucleic Acids Res (2008) 1.18

Generation of rho0 cells utilizing a mitochondrially targeted restriction endonuclease and comparative analyses. Nucleic Acids Res (2008) 1.17

Distinct structural features of TFAM drive mitochondrial DNA packaging versus transcriptional activation. Nat Commun (2014) 1.16

TWINKLE is an essential mitochondrial helicase required for synthesis of nascent D-loop strands and complete mtDNA replication. Hum Mol Genet (2013) 1.16

Expression of a family of noncoding mitochondrial RNAs distinguishes normal from cancer cells. Proc Natl Acad Sci U S A (2009) 1.15

A hybrid G-quadruplex structure formed between RNA and DNA explains the extraordinary stability of the mitochondrial R-loop. Nucleic Acids Res (2012) 1.15

Effect of aging on 5-hydroxymethylcytosine in brain mitochondria. Neurobiol Aging (2012) 1.15

Native R-loops persist throughout the mouse mitochondrial DNA genome. J Biol Chem (2008) 1.14

Biophysical characterizations of human mitochondrial transcription factor A and its binding to tumor suppressor p53. Nucleic Acids Res (2009) 1.13

Estrogen actions on mitochondria--physiological and pathological implications. Mol Cell Endocrinol (2008) 1.11

Sensitivity of mitochondrial transcription and resistance of RNA polymerase II dependent nuclear transcription to antiviral ribonucleosides. PLoS Pathog (2012) 1.11

Mitochondrial biogenesis and turnover. Cell Calcium (2008) 1.10

Evolution of the mitochondrial genome in mammals living at high altitude: new insights from a study of the tribe Caprini (Bovidae, Antilopinae). J Mol Evol (2009) 1.09

Mitochondrial and nuclear accumulation of the transcription factor ATFS-1 promotes OXPHOS recovery during the UPR(mt). Mol Cell (2015) 1.09

Identification of multiple rate-limiting steps during the human mitochondrial transcription cycle in vitro. J Biol Chem (2010) 1.09

Mild heat stress induces mitochondrial biogenesis in C2C12 myotubes. J Appl Physiol (1985) (2011) 1.08

Effect of valproic acid on mitochondrial epigenetics. Eur J Pharmacol (2012) 1.08

Expression of a novel non-coding mitochondrial RNA in human proliferating cells. Nucleic Acids Res (2007) 1.07

The bicoid stability factor controls polyadenylation and expression of specific mitochondrial mRNAs in Drosophila melanogaster. PLoS Genet (2011) 1.06

Paradoxical effect of mitochondrial respiratory chain impairment on insulin signaling and glucose transport in adipose cells. J Biol Chem (2008) 1.05

Somatic mtDNA mutation spectra in the aging human putamen. PLoS Genet (2013) 1.04

LRP130 protein remodels mitochondria and stimulates fatty acid oxidation. J Biol Chem (2011) 1.03

Mitochondrial DNA deletions are associated with non-B DNA conformations. Nucleic Acids Res (2012) 1.03

Genotype-dependent variation of mitochondrial transcriptional profiles in interpopulation hybrids. Proc Natl Acad Sci U S A (2008) 1.03

MTERF3 regulates mitochondrial ribosome biogenesis in invertebrates and mammals. PLoS Genet (2013) 1.01

Mitochondrial DNA and inflammatory diseases. Hum Genet (2011) 1.01

Mitochondrial dysfunction and cancer metastasis. J Bioenerg Biomembr (2012) 1.00

Keeping mtDNA in shape between generations. PLoS Genet (2014) 0.98

Maintenance of respiratory chain function in mouse hearts with severely impaired mtDNA transcription. Nucleic Acids Res (2010) 0.96

Structure of the human MTERF4-NSUN4 protein complex that regulates mitochondrial ribosome biogenesis. Proc Natl Acad Sci U S A (2012) 0.96

Role of protein phosphatases and mitochondria in the neuroprotective effects of estrogens. Front Neuroendocrinol (2009) 0.96

Dynamic regulation of genes involved in mitochondrial DNA replication and transcription during mouse brown fat cell differentiation and recruitment. PLoS One (2009) 0.95

Molecular hydrogen: new antioxidant and anti-inflammatory therapy for rheumatoid arthritis and related diseases. Curr Pharm Des (2013) 0.95

Mitochondrial DNA: A Blind Spot in Neuroepigenetics. Biomol Concepts (2012) 0.95

RNA-level unscrambling of fragmented genes in Diplonema mitochondria. RNA Biol (2013) 0.95

The mitochondrial DNA helicase TWINKLE can assemble on a closed circular template and support initiation of DNA synthesis. Nucleic Acids Res (2011) 0.94

The dynamics of the mitochondrial organelle as a potential therapeutic target. J Cereb Blood Flow Metab (2012) 0.94

Nuclear respiratory factor 2 induces the expression of many but not all human proteins acting in mitochondrial DNA transcription and replication. J Biol Chem (2009) 0.94

Coenzyme Q10 inhibits glutamate excitotoxicity and oxidative stress-mediated mitochondrial alteration in a mouse model of glaucoma. Invest Ophthalmol Vis Sci (2014) 0.94

Inhibition of cyclophilin D by cyclosporin A promotes retinal ganglion cell survival by preventing mitochondrial alteration in ischemic injury. Cell Death Dis (2014) 0.93

Brimonidine blocks glutamate excitotoxicity-induced oxidative stress and preserves mitochondrial transcription factor a in ischemic retinal injury. PLoS One (2012) 0.93

Loss of mitochondrial DNA under genotoxic stress conditions in the absence of the yeast DNA helicase Pif1p occurs independently of the DNA helicase Rrm3p. Mol Genet Genomics (2009) 0.93

Mitochondrial disease in childhood: mtDNA encoded. Neurotherapeutics (2013) 0.93

Animal models of mitochondrial DNA transactions in disease and ageing. Exp Gerontol (2010) 0.93

Human mitochondrial RNA polymerase: evaluation of the single-nucleotide-addition cycle on synthetic RNA/DNA scaffolds. Biochemistry (2011) 0.92

The amino terminal extension of mammalian mitochondrial RNA polymerase ensures promoter specific transcription initiation. Nucleic Acids Res (2014) 0.92

Application of fractional mass for the identification of peptide-oligonucleotide cross-links by mass spectrometry. J Mass Spectrom (2008) 0.91

Nuclear transcription factors in mammalian mitochondria. Genome Biol (2010) 0.91

Advancing animal models of human type 1 diabetes by engraftment of functional human tissues in immunodeficient mice. Cold Spring Harb Perspect Med (2012) 0.91

R-loops in proliferating cells but not in the brain: implications for AOA2 and other autosomal recessive ataxias. PLoS One (2014) 0.90

Mouse models of oxidative phosphorylation defects: powerful tools to study the pathobiology of mitochondrial diseases. Biochim Biophys Acta (2008) 0.90

Adaptation of topoisomerase I paralogs to nuclear and mitochondrial DNA. Nucleic Acids Res (2009) 0.90

Defects of mitochondrial DNA replication. J Child Neurol (2014) 0.90

Negative regulation of mitochondrial transcription by mitochondrial topoisomerase I. Nucleic Acids Res (2013) 0.90

Prevalent coordination of mitochondrial DNA transcription and initiation of replication with the cell cycle. Nucleic Acids Res (2013) 0.90

Ablation of TSC2 enhances insulin secretion by increasing the number of mitochondria through activation of mTORC1. PLoS One (2011) 0.89

Deficiency in repair of the mitochondrial genome sensitizes proliferating myoblasts to oxidative damage. PLoS One (2013) 0.89

Onset and organ specificity of Tk2 deficiency depends on Tk1 down-regulation and transcriptional compensation. Hum Mol Genet (2010) 0.89

Gamma rays induce a p53-independent mitochondrial biogenesis that is counter-regulated by HIF1α. Cell Death Dis (2013) 0.89

Oxidative stress during mitochondrial biogenesis compromises mtDNA integrity in growing hearts and induces a global DNA repair response. Nucleic Acids Res (2012) 0.88

The interface of transcription and DNA replication in the mitochondria. Biochim Biophys Acta (2011) 0.88

Mechanisms linking mtDNA damage and aging. Free Radic Biol Med (2015) 0.88

Replication factors transiently associate with mtDNA at the mitochondrial inner membrane to facilitate replication. Nucleic Acids Res (2013) 0.87

Premature ageing in mice expressing defective mitochondrial DNA polymerase. Nature (2004) 11.59

Mitochondrial transcription factors B1 and B2 activate transcription of human mtDNA. Nat Genet (2002) 4.48

Mitochondrial transcription factor A regulates mtDNA copy number in mammals. Hum Mol Genet (2004) 4.11

Somatic mtDNA mutations cause aging phenotypes without affecting reactive oxygen species production. Proc Natl Acad Sci U S A (2005) 3.42

Strong purifying selection in transmission of mammalian mitochondrial DNA. PLoS Biol (2008) 3.38

Proteolytic processing of OPA1 links mitochondrial dysfunction to alterations in mitochondrial morphology. J Biol Chem (2006) 3.36

Methylation of 12S rRNA is necessary for in vivo stability of the small subunit of the mammalian mitochondrial ribosome. Cell Metab (2009) 3.06

RNA Pol II subunit Rpb7 promotes centromeric transcription and RNAi-directed chromatin silencing. Genes Dev (2005) 2.99

Random point mutations with major effects on protein-coding genes are the driving force behind premature aging in mtDNA mutator mice. Cell Metab (2009) 2.96

Germline mitochondrial DNA mutations aggravate ageing and can impair brain development. Nature (2013) 2.90

Progressive parkinsonism in mice with respiratory-chain-deficient dopamine neurons. Proc Natl Acad Sci U S A (2007) 2.77

MTERF3 is a negative regulator of mammalian mtDNA transcription. Cell (2007) 2.76

The role of mitochondria in aging. J Clin Invest (2013) 2.71

Increased mitochondrial mass in mitochondrial myopathy mice. Proc Natl Acad Sci U S A (2002) 2.38

A unified nomenclature for protein subunits of mediator complexes linking transcriptional regulators to RNA polymerase II. Mol Cell (2004) 2.27

Somatic progenitor cell vulnerability to mitochondrial DNA mutagenesis underlies progeroid phenotypes in Polg mutator mice. Cell Metab (2012) 2.21

The yeast Mediator complex and its regulation. Trends Biochem Sci (2005) 2.15

Architectural role of mitochondrial transcription factor A in maintenance of human mitochondrial DNA. Mol Cell Biol (2004) 1.97

Variation in germline mtDNA heteroplasmy is determined prenatally but modified during subsequent transmission. Nat Genet (2012) 1.94

The cyclin-dependent kinase 8 module sterically blocks Mediator interactions with RNA polymerase II. Proc Natl Acad Sci U S A (2006) 1.91

Super-resolution microscopy reveals that mammalian mitochondrial nucleoids have a uniform size and frequently contain a single copy of mtDNA. Proc Natl Acad Sci U S A (2011) 1.90

LRPPRC is necessary for polyadenylation and coordination of translation of mitochondrial mRNAs. EMBO J (2011) 1.89

Nurr1 is required for maintenance of maturing and adult midbrain dopamine neurons. J Neurosci (2009) 1.89

Mitochondrial transcription and its regulation in mammalian cells. Trends Biochem Sci (2007) 1.88

Genome-wide occupancy profile of mediator and the Srb8-11 module reveals interactions with coding regions. Mol Cell (2006) 1.86

Impaired mitochondrial transport and Parkin-independent degeneration of respiratory chain-deficient dopamine neurons in vivo. Proc Natl Acad Sci U S A (2011) 1.82

The mitochondrial RNA polymerase contributes critically to promoter specificity in mammalian cells. EMBO J (2004) 1.81

MTERF4 regulates translation by targeting the methyltransferase NSUN4 to the mammalian mitochondrial ribosome. Cell Metab (2011) 1.68

Human mitochondrial transcription revisited: only TFAM and TFB2M are required for transcription of the mitochondrial genes in vitro. J Biol Chem (2010) 1.67

Mitochondrial DNA mutations in disease and aging. J Cell Biol (2011) 1.67

Purifying selection of mtDNA and its implications for understanding evolution and mitochondrial disease. Nat Rev Genet (2008) 1.60

Ultra-deep sequencing of mouse mitochondrial DNA: mutational patterns and their origins. PLoS Genet (2011) 1.60

A genome-wide role for CHD remodelling factors and Nap1 in nucleosome disassembly. EMBO J (2007) 1.59

Mitochondrial RNA polymerase is needed for activation of the origin of light-strand DNA replication. Mol Cell (2010) 1.53

A switch in metabolism precedes increased mitochondrial biogenesis in respiratory chain-deficient mouse hearts. Proc Natl Acad Sci U S A (2004) 1.53

Human mitochondrial RNA polymerase primes lagging-strand DNA synthesis in vitro. Proc Natl Acad Sci U S A (2008) 1.52

High brain lactate is a hallmark of aging and caused by a shift in the lactate dehydrogenase A/B ratio. Proc Natl Acad Sci U S A (2010) 1.52

Mitofusin 2 is necessary for striatal axonal projections of midbrain dopamine neurons. Hum Mol Genet (2012) 1.42

The transcription machinery in mammalian mitochondria. Biochim Biophys Acta (2004) 1.41

Conserved sequence box II directs transcription termination and primer formation in mitochondria. J Biol Chem (2006) 1.40

Mammalian transcription factor A is a core component of the mitochondrial transcription machinery. Proc Natl Acad Sci U S A (2012) 1.37

TRAP230/ARC240 and TRAP240/ARC250 Mediator subunits are functionally conserved through evolution. Proc Natl Acad Sci U S A (2003) 1.33

A family of putative transcription termination factors shared amongst metazoans and plants. Curr Genet (2005) 1.33

Adipose-specific deletion of TFAM increases mitochondrial oxidation and protects mice against obesity and insulin resistance. Cell Metab (2012) 1.32

Increased mitochondrial Ca2+ and decreased sarcoplasmic reticulum Ca2+ in mitochondrial myopathy. Hum Mol Genet (2008) 1.29

Respiratory chain dysfunction in skeletal muscle does not cause insulin resistance. Biochem Biophys Res Commun (2006) 1.27

SOD2 overexpression: enhanced mitochondrial tolerance but absence of effect on UCP activity. EMBO J (2005) 1.26

G-quadruplex structures in RNA stimulate mitochondrial transcription termination and primer formation. Proc Natl Acad Sci U S A (2010) 1.21

Impaired nigrostriatal function precedes behavioral deficits in a genetic mitochondrial model of Parkinson's disease. FASEB J (2011) 1.19

Reactive oxygen species and fatigue-induced prolonged low-frequency force depression in skeletal muscle fibres of rats, mice and SOD2 overexpressing mice. J Physiol (2007) 1.18

Molecular phylogenetics of ascomycotal adhesins--a novel family of putative cell-surface adhesive proteins in fission yeasts. Fungal Genet Biol (2007) 1.16

TWINKLE is an essential mitochondrial helicase required for synthesis of nascent D-loop strands and complete mtDNA replication. Hum Mol Genet (2013) 1.16

A hybrid G-quadruplex structure formed between RNA and DNA explains the extraordinary stability of the mitochondrial R-loop. Nucleic Acids Res (2012) 1.15

Adenosine kinase deficiency disrupts the methionine cycle and causes hypermethioninemia, encephalopathy, and abnormal liver function. Am J Hum Genet (2011) 1.14

The structural and functional role of Med5 in the yeast Mediator tail module. J Biol Chem (2005) 1.10

Mediator influences Schizosaccharomyces pombe RNA polymerase II-dependent transcription in vitro. J Biol Chem (2003) 1.09

MTERF2 is a nucleoid component in mammalian mitochondria. Biochim Biophys Acta (2009) 1.09

Protein sliding and DNA denaturation are essential for DNA organization by human mitochondrial transcription factor A. Nat Commun (2012) 1.07

The bicoid stability factor controls polyadenylation and expression of specific mitochondrial mRNAs in Drosophila melanogaster. PLoS Genet (2011) 1.06

Altered dopamine metabolism and increased vulnerability to MPTP in mice with partial deficiency of mitochondrial complex I in dopamine neurons. Hum Mol Genet (2011) 1.06

In vivo mutagenesis reveals that OriL is essential for mitochondrial DNA replication. EMBO Rep (2012) 1.06

Two novel mutations in thymidine kinase-2 cause early onset fatal encephalomyopathy and severe mtDNA depletion. Neuromuscul Disord (2010) 1.06

The multitalented Mediator complex. Trends Biochem Sci (2013) 1.04

MTERF1 binds mtDNA to prevent transcriptional interference at the light-strand promoter but is dispensable for rRNA gene transcription regulation. Cell Metab (2013) 1.03

LRPPRC is a mitochondrial matrix protein that is conserved in metazoans. Biochem Biophys Res Commun (2010) 1.03

The mitochondrial electron transport chain is dispensable for proliferation and differentiation of epidermal progenitor cells. Stem Cells (2011) 1.03

A common variant in TFB1M is associated with reduced insulin secretion and increased future risk of type 2 diabetes. Cell Metab (2011) 1.03

mtDNA makes a U-turn for the mitochondrial nucleoid. Trends Cell Biol (2013) 1.02

Breeding and genotyping of Tfam conditional knockout mice. Methods Mol Biol (2002) 1.02

MTERF3 regulates mitochondrial ribosome biogenesis in invertebrates and mammals. PLoS Genet (2013) 1.01

Loss of LRPPRC causes ATP synthase deficiency. Hum Mol Genet (2014) 1.01

The human mitochondrial transcription termination factor (mTERF) is fully active in vitro in the non-phosphorylated form. J Biol Chem (2005) 1.00

The Soh1/MED31 protein is an ancient component of Schizosaccharomyces pombe and Saccharomyces cerevisiae Mediator. J Biol Chem (2004) 1.00

Mitochondrial dysfunction in mammalian ageing. Novartis Found Symp (2007) 1.00

Role of human DNA glycosylase Nei-like 2 (NEIL2) and single strand break repair protein polynucleotide kinase 3'-phosphatase in maintenance of mitochondrial genome. J Biol Chem (2011) 0.98

Sequence-specific stalling of DNA polymerase γ and the effects of mutations causing progressive ophthalmoplegia. Hum Mol Genet (2011) 0.97

Understanding aging: revealing order out of chaos. Biochim Biophys Acta (2004) 0.97

Somatic mtDNA mutations cause progressive hearing loss in the mouse. Exp Cell Res (2007) 0.97

Secondary metabolic effects in complex I deficiency. Ann Neurol (2005) 0.97

Two conserved modules of Schizosaccharomyces pombe Mediator regulate distinct cellular pathways. Nucleic Acids Res (2008) 0.96

The classical srb4-138 mutant allele causes dissociation of yeast Mediator. Biochem Biophys Res Commun (2006) 0.96

Structure of mitochondrial transcription termination factor 3 reveals a novel nucleic acid-binding domain. Biochem Biophys Res Commun (2010) 0.96

Maintenance of respiratory chain function in mouse hearts with severely impaired mtDNA transcription. Nucleic Acids Res (2010) 0.96

Structure of the human MTERF4-NSUN4 protein complex that regulates mitochondrial ribosome biogenesis. Proc Natl Acad Sci U S A (2012) 0.96

AFG3L2 supports mitochondrial protein synthesis and Purkinje cell survival. J Clin Invest (2012) 0.95