Accessorizing the human mitochondrial transcription machinery.

MicroRNA directly enhances mitochondrial translation during muscle differentiation. Cell (2014) 2.14

Mitochondrial retrograde signaling at the crossroads of tumor bioenergetics, genetics and epigenetics. Mitochondrion (2013) 1.17

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

RNA-DNA differences in human mitochondria restore ancestral form of 16S ribosomal RNA. Genome Res (2013) 0.99

Yeast as a system for modeling mitochondrial disease mechanisms and discovering therapies. Dis Model Mech (2015) 0.98

The genetics and pathology of mitochondrial disease. J Pathol (2016) 0.92

Pathological ribonuclease H1 causes R-loop depletion and aberrant DNA segregation in mitochondria. Proc Natl Acad Sci U S A (2016) 0.84

A model for transcription initiation in human mitochondria. Nucleic Acids Res (2015) 0.84

tRNA biology in mitochondria. Int J Mol Sci (2015) 0.84

Transcription factors bind negatively selected sites within human mtDNA genes. Genome Biol Evol (2014) 0.82

Cytonuclear Interactions in the Evolution of Animal Mitochondrial tRNA Metabolism. Genome Biol Evol (2015) 0.80

Differential contribution of the mitochondrial translation pathway to the survival of diffuse large B-cell lymphoma subsets. Cell Death Differ (2016) 0.78

Echinochrome a increases mitochondrial mass and function by modulating mitochondrial biogenesis regulatory genes. Mar Drugs (2014) 0.78

Mitochondrial transcription factor A regulation of mitochondrial degeneration in experimental diabetic neuropathy. Am J Physiol Endocrinol Metab (2015) 0.78

Mitochondrial Ribosomal Protein L12 Is Required for POLRMT Stability and Exists as Two Forms Generated by Alternative Proteolysis during Import. J Biol Chem (2015) 0.77

Population-level expression variability of mitochondrial DNA-encoded genes in humans. Eur J Hum Genet (2014) 0.76

Mitochondrial dysfunction and mitochondrial dynamics-The cancer connection. Biochim Biophys Acta (2017) 0.76

Mitochondrial DNA in innate immune responses and inflammatory pathology. Nat Rev Immunol (2017) 0.75

Fancd2 in vivo interaction network reveals a non-canonical role in mitochondrial function. Sci Rep (2017) 0.75

Function of specialized regulatory proteins and signaling pathways in exercise-induced muscle mitochondrial biogenesis. Integr Med Res (2016) 0.75

Experimental mitochondria-targeted DNA methylation identifies GpC methylation, not CpG methylation, as potential regulator of mitochondrial gene expression. Sci Rep (2017) 0.75

A comparison among the tissue-specific effects of aging and calorie restriction on TFAM amount and TFAM-binding activity to mtDNA in rat. Biochim Biophys Acta (2014) 0.75

A mitochondrial paradigm of metabolic and degenerative diseases, aging, and cancer: a dawn for evolutionary medicine. Annu Rev Genet (2005) 16.79

tRNA punctuation model of RNA processing in human mitochondria. Nature (1981) 8.48

Identification of a gene causing human cytochrome c oxidase deficiency by integrative genomics. Proc Natl Acad Sci U S A (2003) 6.87

Mitochondrial DNA maintenance in vertebrates. Annu Rev Biochem (1997) 4.96

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

Similarity of human mitochondrial transcription factor 1 to high mobility group proteins. Science (1991) 4.26

Yeast mitochondrial RNA polymerase is homologous to those encoded by bacteriophages T3 and T7. Cell (1987) 3.83

Initiation and beyond: multiple functions of the human mitochondrial transcription machinery. Mol Cell (2006) 3.16

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

Priming of human mitochondrial DNA replication occurs at the light-strand promoter. Proc Natl Acad Sci U S A (1985) 3.05

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

DNA wrapping and bending by a mitochondrial high mobility group-like transcriptional activator protein. J Biol Chem (1992) 2.64

The pattern of transcription of the human mitochondrial rRNA genes reveals two overlapping transcription units. Cell (1983) 2.60

Calorie restriction and SIRT3 trigger global reprogramming of the mitochondrial protein acetylome. Mol Cell (2012) 2.54

Promoter selection in human mitochondria involves binding of a transcription factor to orientation-independent upstream regulatory elements. Cell (1987) 2.44

The mitochondrial transcription factor TFAM coordinates the assembly of multiple DNA molecules into nucleoid-like structures. Mol Biol Cell (2007) 2.42

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

Addition of a 29 residue carboxyl-terminal tail converts a simple HMG box-containing protein into a transcriptional activator. J Mol Biol (1995) 2.17

Identification of the gene encoding the human mitochondrial RNA polymerase (h-mtRPOL) by cyberscreening of the Expressed Sequence Tags database. Hum Mol Genet (1997) 2.15

Termination factor-mediated DNA loop between termination and initiation sites drives mitochondrial rRNA synthesis. Cell (2005) 2.01

Termination of transcription in human mitochondria: identification and purification of a DNA binding protein factor that promotes termination. Cell (1989) 1.97

Helix unwinding and base flipping enable human MTERF1 to terminate mitochondrial transcription. Cell (2010) 1.93

LRPPRC and SLIRP interact in a ribonucleoprotein complex that regulates posttranscriptional gene expression in mitochondria. Mol Biol Cell (2010) 1.93

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

LRP130, a pentatricopeptide motif protein with a noncanonical RNA-binding domain, is bound in vivo to mitochondrial and nuclear RNAs. Mol Cell Biol (2003) 1.89

Human mitochondrial transcription factor B1 methylates ribosomal RNA at a conserved stem-loop. Nat Genet (2002) 1.89

A transcription factor required for promoter recognition by human mitochondrial RNA polymerase. Accurate initiation at the heavy- and light-strand promoters dissected and reconstituted in vitro. J Biol Chem (1985) 1.86

The role of the LRPPRC (leucine-rich pentatricopeptide repeat cassette) gene in cytochrome oxidase assembly: mutation causes lowered levels of COX (cytochrome c oxidase) I and COX III mRNA. Biochem J (2004) 1.85

A human mitochondrial transcription factor is related to RNA adenine methyltransferases and binds S-adenosylmethionine. Mol Cell Biol (2002) 1.85

Structure of human mitochondrial RNA polymerase. Nature (2011) 1.82

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

Human mitochondrial transcription factor B1 interacts with the C-terminal activation region of h-mtTFA and stimulates transcription independently of its RNA methyltransferase activity. Mol Cell Biol (2003) 1.80

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

Mitochondrial transcription initiation. Variation and conservation. J Biol Chem (1993) 1.74

In vitro replication of human mitochondrial DNA: accurate initiation at the origin of light-strand synthesis. Cell (1985) 1.72

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

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

Modular architecture of the bacteriophage T7 primase couples RNA primer synthesis to DNA synthesis. Mol Cell (2003) 1.61

Human mitochondrial ribosomal protein MRPL12 interacts directly with mitochondrial RNA polymerase to modulate mitochondrial gene expression. J Biol Chem (2007) 1.54

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

Human mitochondrial transcription factor A induces a U-turn structure in the light strand promoter. Nat Struct Mol Biol (2011) 1.51

P53 physically interacts with mitochondrial transcription factor A and differentially regulates binding to damaged DNA. Cancer Res (2003) 1.44

p53 improves aerobic exercise capacity and augments skeletal muscle mitochondrial DNA content. Circ Res (2009) 1.39

Mitochondrial Lon protease regulates mitochondrial DNA copy number and transcription by selective degradation of mitochondrial transcription factor A (TFAM). Proc Natl Acad Sci U S A (2010) 1.38

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

The MTERF family proteins: mitochondrial transcription regulators and beyond. Biochim Biophys Acta (2009) 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

The human mitochondrial replication fork in health and disease. Biochim Biophys Acta (2010) 1.33

Retracted mTERF2 regulates oxidative phosphorylation by modulating mtDNA transcription. Cell Metab (2009) 1.31

Coupling the mitochondrial transcription machinery to human disease. Trends Genet (2004) 1.31

LRPPRC/SLIRP suppresses PNPase-mediated mRNA decay and promotes polyadenylation in human mitochondria. Nucleic Acids Res (2012) 1.29

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

The Drosophila mitochondrial ribosomal protein mRpL12 is required for Cyclin D/Cdk4-driven growth. EMBO J (2005) 1.25

Mitochondrial protein import: from transport pathways to an integrated network. Trends Biochem Sci (2011) 1.23

Evidence for an early gene duplication event in the evolution of the mitochondrial transcription factor B family and maintenance of rRNA methyltransferase activity in human mtTFB1 and mtTFB2. J Mol Evol (2006) 1.23

Mitochondrial DNA nucleoid structure. Biochim Biophys Acta (2011) 1.21

Mitochondrial DNA replication and repair: all a flap. Trends Biochem Sci (2009) 1.20

Transcriptional activation by mitochondrial transcription factor A involves preferential distortion of promoter DNA. Nucleic Acids Res (2011) 1.15

Leucine-rich pentatricopeptide-repeat containing protein regulates mitochondrial transcription. Biochemistry (2010) 1.13

Human mitochondrial mTERF wraps around DNA through a left-handed superhelical tandem repeat. Nat Struct Mol Biol (2010) 1.12

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

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

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

Elucidation of separate, but collaborative functions of the rRNA methyltransferase-related human mitochondrial transcription factors B1 and B2 in mitochondrial biogenesis reveals new insight into maternally inherited deafness. Hum Mol Genet (2009) 1.07

Multi-tasking: nuclear transcription factors with novel roles in the mitochondria. Trends Cell Biol (2012) 1.05

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

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

Mitochondrial ribosomal protein L12 selectively associates with human mitochondrial RNA polymerase to activate transcription. Proc Natl Acad Sci U S A (2011) 1.02

A delayed-early response nuclear gene encoding MRPL12, the mitochondrial homologue to the bacterial translational regulator L7/L12 protein. J Biol Chem (1996) 0.99

PPR (pentatricopeptide repeat) proteins in mammals: important aids to mitochondrial gene expression. Biochem J (2008) 0.98

LRPPRC mutations cause a phenotypically distinct form of Leigh syndrome with cytochrome c oxidase deficiency. J Med Genet (2011) 0.98

Transcription-independent role for human mitochondrial RNA polymerase in mitochondrial ribosome biogenesis. Nucleic Acids Res (2013) 0.96

The role of mammalian PPR domain proteins in the regulation of mitochondrial gene expression. Biochim Biophys Acta (2011) 0.95

Human Cockayne syndrome B protein reciprocally communicates with mitochondrial proteins and promotes transcriptional elongation. Nucleic Acids Res (2012) 0.94

Transcription from the second heavy-strand promoter of human mtDNA is repressed by transcription factor A in vitro. Proc Natl Acad Sci U S A (2012) 0.93

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

LRPPRC mutation suppresses cytochrome oxidase activity by altering mitochondrial RNA transcript stability in a mouse model. Biochem J (2012) 0.92

Overexpression of MTERFD1 or MTERFD3 impairs the completion of mitochondrial DNA replication. Mol Biol Rep (2010) 0.92

Transcriptional requirements of the distal heavy-strand promoter of mtDNA. Proc Natl Acad Sci U S A (2012) 0.89

The core human mitochondrial transcription initiation complex: It only takes two to tango. Transcription (2011) 0.87

Reduced TOR signaling extends chronological life span via increased respiration and upregulation of mitochondrial gene expression. Cell Metab (2007) 4.59

TLR signalling augments macrophage bactericidal activity through mitochondrial ROS. Nature (2011) 4.16

Absence of autophagy results in reactive oxygen species-dependent amplification of RLR signaling. Proc Natl Acad Sci U S A (2009) 3.84

Mitochondria in innate immune responses. Nat Rev Immunol (2011) 3.31

Initiation and beyond: multiple functions of the human mitochondrial transcription machinery. Mol Cell (2006) 3.16

Regulation of yeast chronological life span by TORC1 via adaptive mitochondrial ROS signaling. Cell Metab (2011) 3.13

Targeted expression of catalase to mitochondria prevents age-associated reductions in mitochondrial function and insulin resistance. Cell Metab (2010) 3.07

Replicative and chronological aging in Saccharomyces cerevisiae. Cell Metab (2012) 2.56

Ataxia-telangiectasia mutated kinase regulates ribonucleotide reductase and mitochondrial homeostasis. J Clin Invest (2007) 2.03

Epigenetic silencing mediates mitochondria stress-induced longevity. Cell Metab (2013) 2.03

Human mitochondrial transcription factor B1 methylates ribosomal RNA at a conserved stem-loop. Nat Genet (2002) 1.89

A human mitochondrial transcription factor is related to RNA adenine methyltransferases and binds S-adenosylmethionine. Mol Cell Biol (2002) 1.85

Extension of chronological life span by reduced TOR signaling requires down-regulation of Sch9p and involves increased mitochondrial OXPHOS complex density. Aging (Albany NY) (2009) 1.82

Human mitochondrial transcription factor B1 interacts with the C-terminal activation region of h-mtTFA and stimulates transcription independently of its RNA methyltransferase activity. Mol Cell Biol (2003) 1.80

Loss of p53 causes mitochondrial DNA depletion and altered mitochondrial reactive oxygen species homeostasis. Biochim Biophys Acta (2009) 1.67

Modulation of mitochondrial transcription in response to mtDNA depletion and repletion in HeLa cells. Nucleic Acids Res (2002) 1.64

Relative abundance of the human mitochondrial transcription system and distinct roles for h-mtTFB1 and h-mtTFB2 in mitochondrial biogenesis and gene expression. Nucleic Acids Res (2007) 1.60

Ancient mtDNA genetic variants modulate mtDNA transcription and replication. PLoS Genet (2009) 1.58

Prohibitin-1 maintains the angiogenic capacity of endothelial cells by regulating mitochondrial function and senescence. J Cell Biol (2008) 1.56

Mitochondrial stress engages E2F1 apoptotic signaling to cause deafness. Cell (2012) 1.56

Human mitochondrial ribosomal protein MRPL12 interacts directly with mitochondrial RNA polymerase to modulate mitochondrial gene expression. J Biol Chem (2007) 1.54

Revisiting the TCA cycle: signaling to tumor formation. Trends Mol Med (2011) 1.51

Defective mitochondrial gene expression results in reactive oxygen species-mediated inhibition of respiration and reduction of yeast life span. Mol Cell Biol (2006) 1.47

The conserved Mec1/Rad53 nuclear checkpoint pathway regulates mitochondrial DNA copy number in Saccharomyces cerevisiae. Mol Biol Cell (2005) 1.45

Mitochondrial respiratory thresholds regulate yeast chronological life span and its extension by caloric restriction. Cell Metab (2012) 1.41

Multiple interactions involving the amino-terminal domain of yeast mtRNA polymerase determine the efficiency of mitochondrial protein synthesis. J Biol Chem (2003) 1.38

Mitochondrial dysfunction due to oxidative mitochondrial DNA damage is reduced through cooperative actions of diverse proteins. Mol Cell Biol (2002) 1.35

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

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

Evidence for an early gene duplication event in the evolution of the mitochondrial transcription factor B family and maintenance of rRNA methyltransferase activity in human mtTFB1 and mtTFB2. J Mol Evol (2006) 1.23

Sls1p is a membrane-bound regulator of transcription-coupled processes involved in Saccharomyces cerevisiae mitochondrial gene expression. Genetics (2002) 1.22

Convergence of multiple signaling pathways is required to coordinately up-regulate mtDNA and mitochondrial biogenesis during T cell activation. Mitochondrion (2007) 1.21

Oxidative DNA damage causes mitochondrial genomic instability in Saccharomyces cerevisiae. Mol Cell Biol (2005) 1.21

Mitochondrial genome instability and ROS enhance intestinal tumorigenesis in APC(Min/+) mice. Am J Pathol (2011) 1.18

A novel mitochondrial protein, Tar1p, is encoded on the antisense strand of the nuclear 25S rDNA. Genes Dev (2002) 1.16

Transcriptional activation by mitochondrial transcription factor A involves preferential distortion of promoter DNA. Nucleic Acids Res (2011) 1.15

Monocyte activation by necrotic cells is promoted by mitochondrial proteins and formyl peptide receptors. Crit Care Med (2009) 1.10

Differential involvement of the related DNA helicases Pif1p and Rrm3p in mtDNA point mutagenesis and stability. Gene (2005) 1.08

Cell cycle- and ribonucleotide reductase-driven changes in mtDNA copy number influence mtDNA Inheritance without compromising mitochondrial gene expression. Cell Cycle (2007) 1.07

Elucidation of separate, but collaborative functions of the rRNA methyltransferase-related human mitochondrial transcription factors B1 and B2 in mitochondrial biogenesis reveals new insight into maternally inherited deafness. Hum Mol Genet (2009) 1.07

Rethinking the mitochondrial theory of aging: the role of mitochondrial gene expression in lifespan determination. Cell Cycle (2007) 1.05

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

Excess ribonucleotide reductase R2 subunits coordinate the S phase checkpoint to facilitate DNA damage repair and recovery from replication stress. Biochem Pharmacol (2006) 1.03

Mitochondrial ribosomal protein L12 selectively associates with human mitochondrial RNA polymerase to activate transcription. Proc Natl Acad Sci U S A (2011) 1.02

Mitochondrial stress signals revise an old aging theory. Cell (2011) 0.97

Transcription-independent role for human mitochondrial RNA polymerase in mitochondrial ribosome biogenesis. Nucleic Acids Res (2013) 0.96

Transcription from the second heavy-strand promoter of human mtDNA is repressed by transcription factor A in vitro. Proc Natl Acad Sci U S A (2012) 0.93

Repression of mitochondrial translation, respiration and a metabolic cycle-regulated gene, SLF1, by the yeast Pumilio-family protein Puf3p. PLoS One (2011) 0.93

Reducing mitochondrial ROS improves disease-related pathology in a mouse model of ataxia-telangiectasia. Mol Ther (2012) 0.92

The core human mitochondrial transcription initiation complex: It only takes two to tango. Transcription (2011) 0.87

Suppression of NLRX1 in chronic obstructive pulmonary disease. J Clin Invest (2015) 0.85

Genomic imprinting at a boundary element flanking the SDHD locus. Hum Mol Genet (2011) 0.84

Aberrant CD8+ T-cell responses and memory differentiation upon viral infection of an ataxia-telangiectasia mouse model driven by hyper-activated Akt and mTORC1 signaling. Am J Pathol (2011) 0.84

Expression of the rDNA-encoded mitochondrial protein Tar1p is stringently controlled and responds differentially to mitochondrial respiratory demand and dysfunction. Curr Genet (2008) 0.84

MKK3 regulates mitochondrial biogenesis and mitophagy in sepsis-induced lung injury. Am J Physiol Lung Cell Mol Physiol (2014) 0.83

Alternative mitochondrial fuel extends life span. Cell Metab (2012) 0.80

Expanding the mitochondrial interactome. Genome Biol (2007) 0.79

Multi-faceted regulation of mitochondria by TOR. Cell Cycle (2009) 0.79

Checkpoint kinase phosphorylation in response to endogenous oxidative DNA damage in repair-deficient stationary-phase Saccharomyces cerevisiae. Mech Ageing Dev (2009) 0.79

Mitochondrial DNA oxidative damage and mutagenesis in Saccharomyces cerevisiae. Methods Mol Biol (2009) 0.76

A "radical" mitochondrial view of autophagy-related pathology. Aging (Albany NY) (2009) 0.76

Stressed-out mitochondria get MAD. Cell Metab (2010) 0.76

Debut of MitoMatters. Mitochondrion (2008) 0.75

Into the wild frontier of mitochondrial disease and aging. Methods (2008) 0.75