Published in Nature on May 27, 2004
A mitochondrial paradigm of metabolic and degenerative diseases, aging, and cancer: a dawn for evolutionary medicine. Annu Rev Genet (2005) 16.79
The hallmarks of aging. Cell (2013) 14.29
Mitochondrial DNA mutations in human disease. Nat Rev Genet (2005) 8.51
Telomere dysfunction induces metabolic and mitochondrial compromise. Nature (2011) 6.36
Mitochondria: in sickness and in health. Cell (2012) 6.05
Signal transduction by reactive oxygen species. J Cell Biol (2011) 5.21
Mitochondrial fusion is required for mtDNA stability in skeletal muscle and tolerance of mtDNA mutations. Cell (2010) 5.14
Linking functional decline of telomeres, mitochondria and stem cells during ageing. Nature (2010) 5.02
Early aging and age-related pathologies in mice deficient in BMAL1, the core componentof the circadian clock. Genes Dev (2006) 4.90
Redox regulation of cell survival. Antioxid Redox Signal (2008) 4.33
Mitochondrial fragmentation in neurodegeneration. Nat Rev Neurosci (2008) 4.32
Decline in skeletal muscle mitochondrial function with aging in humans. Proc Natl Acad Sci U S A (2005) 4.03
Neural mechanisms of ageing and cognitive decline. Nature (2010) 3.59
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
Mitochondria--a nexus for aging, calorie restriction, and sirtuins? Cell (2008) 3.38
The fidelity of DNA synthesis by eukaryotic replicative and translesion synthesis polymerases. Cell Res (2008) 3.15
p63 deficiency activates a program of cellular senescence and leads to accelerated aging. Genes Dev (2005) 3.02
Mitochondrial oxidative stress mediates angiotensin II-induced cardiac hypertrophy and Galphaq overexpression-induced heart failure. Circ Res (2011) 2.99
Premature aging-like phenotype in fibroblast growth factor 23 null mice is a vitamin D-mediated process. FASEB J (2006) 2.99
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
Amyloid beta, mitochondrial dysfunction and synaptic damage: implications for cognitive decline in aging and Alzheimer's disease. Trends Mol Med (2008) 2.74
The role of mitochondria in aging. J Clin Invest (2013) 2.71
The role of mitochondria in the pathogenesis of type 2 diabetes. Endocr Rev (2010) 2.59
Structural insight into processive human mitochondrial DNA synthesis and disease-related polymerase mutations. Cell (2009) 2.51
Mitochondrial Dysfunction Induces Senescence with a Distinct Secretory Phenotype. Cell Metab (2015) 2.48
Mitochondrial DNA-deletion mutations accumulate intracellularly to detrimental levels in aged human skeletal muscle fibers. Am J Hum Genet (2006) 2.43
Universal heteroplasmy of human mitochondrial DNA. Hum Mol Genet (2012) 2.39
Mitochondrial dysfunction in Parkinson's disease: molecular mechanisms and pathophysiological consequences. EMBO J (2012) 2.36
The role of mitochondrial DNA mutations in mammalian aging. PLoS Genet (2007) 2.25
Novel role of p53 in maintaining mitochondrial genetic stability through interaction with DNA Pol gamma. EMBO J (2005) 2.25
Mitochondrial DNA polymerase W748S mutation: a common cause of autosomal recessive ataxia with ancient European origin. Am J Hum Genet (2005) 2.23
The aging stress response. Mol Cell (2010) 2.19
The role of AMP-activated protein kinase in mitochondrial biogenesis. J Physiol (2006) 2.19
The metabolic syndrome resulting from a knockout of the NEIL1 DNA glycosylase. Proc Natl Acad Sci U S A (2006) 2.17
Aging, rejuvenation, and epigenetic reprogramming: resetting the aging clock. Cell (2012) 2.15
The role of mitochondrial DNA copy number in mammalian fertility. Biol Reprod (2010) 2.15
Decreased mtDNA, oxidative stress, cardiomyopathy, and death from transgenic cardiac targeted human mutant polymerase gamma. Lab Invest (2006) 2.14
Meta-analyses identify 13 loci associated with age at menopause and highlight DNA repair and immune pathways. Nat Genet (2012) 2.12
Effects of hypothalamic neurodegeneration on energy balance. PLoS Biol (2005) 2.11
DNA damage, cellular senescence and organismal ageing: causal or correlative? Nucleic Acids Res (2007) 2.09
Somatic mitochondrial DNA mutations in early Parkinson and incidental Lewy body disease. Ann Neurol (2012) 2.08
Energetics, epigenetics, mitochondrial genetics. Mitochondrion (2009) 2.08
Rb intrinsically promotes erythropoiesis by coupling cell cycle exit with mitochondrial biogenesis. Genes Dev (2008) 2.05
New insights into the role of mitochondria in aging: mitochondrial dynamics and more. J Cell Sci (2010) 2.03
A cluster of metabolic defects caused by mutation in a mitochondrial tRNA. Science (2004) 1.95
Identification of a protein mediating respiratory supercomplex stability. Cell Metab (2012) 1.95
Oxidative stress, mitochondrial dysfunction, and aging. J Signal Transduct (2011) 1.94
Variation in germline mtDNA heteroplasmy is determined prenatally but modified during subsequent transmission. Nat Genet (2012) 1.94
Mitochondrial aging is accelerated by anti-retroviral therapy through the clonal expansion of mtDNA mutations. Nat Genet (2011) 1.92
NF-κB inhibition delays DNA damage-induced senescence and aging in mice. J Clin Invest (2012) 1.91
Resistance exercise reverses aging in human skeletal muscle. PLoS One (2007) 1.90
Mutant mitochondrial helicase Twinkle causes multiple mtDNA deletions and a late-onset mitochondrial disease in mice. Proc Natl Acad Sci U S A (2005) 1.88
The pathophysiology of mitochondrial disease as modeled in the mouse. Genes Dev (2009) 1.87
Proteins of nucleotide and base excision repair pathways interact in mitochondria to protect from loss of subcutaneous fat, a hallmark of aging. J Exp Med (2010) 1.86
Targeting mitochondrial dysfunction: role for PINK1 and Parkin in mitochondrial quality control. Antioxid Redox Signal (2011) 1.82
Endurance exercise rescues progeroid aging and induces systemic mitochondrial rejuvenation in mtDNA mutator mice. Proc Natl Acad Sci U S A (2011) 1.81
Expression and maintenance of mitochondrial DNA: new insights into human disease pathology. Am J Pathol (2008) 1.78
Axis of ageing: telomeres, p53 and mitochondria. Nat Rev Mol Cell Biol (2012) 1.74
Mechanisms that regulate stem cell aging and life span. Cell Stem Cell (2013) 1.73
Oxidative stress induces degradation of mitochondrial DNA. Nucleic Acids Res (2009) 1.73
The influence of genes on the aging process of mice: a statistical assessment of the genetics of aging. Genetics (2004) 1.72
Accumulation of mitochondrial DNA deletion mutations in aged muscle fibers: evidence for a causal role in muscle fiber loss. J Gerontol A Biol Sci Med Sci (2007) 1.69
The cellular, developmental and population-genetic determinants of mutation-rate evolution. Genetics (2008) 1.69
Expression of catalytic mutants of the mtDNA helicase Twinkle and polymerase POLG causes distinct replication stalling phenotypes. Nucleic Acids Res (2007) 1.69
Mitochondrial DNA mutations in disease and aging. J Cell Biol (2011) 1.67
DNA precursor asymmetries in mammalian tissue mitochondria and possible contribution to mutagenesis through reduced replication fidelity. Proc Natl Acad Sci U S A (2005) 1.67
The mitochondrial impairment, oxidative stress and neurodegeneration connection: reality or just an attractive hypothesis? Trends Neurosci (2008) 1.65
Mitochondrial turnover and aging of long-lived postmitotic cells: the mitochondrial-lysosomal axis theory of aging. Antioxid Redox Signal (2010) 1.64
A perspective on the determination of mitochondrial biogenesis. Am J Physiol Endocrinol Metab (2011) 1.63
SIRT6 in DNA repair, metabolism and ageing. J Intern Med (2008) 1.61
Cisd2 deficiency drives premature aging and causes mitochondria-mediated defects in mice. Genes Dev (2009) 1.61
Correlates of substitution rate variation in mammalian protein-coding sequences. BMC Evol Biol (2008) 1.60
Ultra-deep sequencing of mouse mitochondrial DNA: mutational patterns and their origins. PLoS Genet (2011) 1.60
The Alzheimer's disease mitochondrial cascade hypothesis. J Alzheimers Dis (2010) 1.60
DNA damage in telomeres and mitochondria during cellular senescence: is there a connection? Nucleic Acids Res (2007) 1.60
Mitochondrial DNA replication and disease: insights from DNA polymerase γ mutations. Cell Mol Life Sci (2010) 1.58
Regulation of mitochondrial biogenesis. Essays Biochem (2010) 1.58
Inflammaging: disturbed interplay between autophagy and inflammasomes. Aging (Albany NY) (2012) 1.58
Dietary and genetic evidence for phosphate toxicity accelerating mammalian aging. FASEB J (2010) 1.58
GRSF1 regulates RNA processing in mitochondrial RNA granules. Cell Metab (2013) 1.57
Mitochondrial dysfunction in cancer. Front Oncol (2013) 1.57
Mitochondrial DNA mutations induce mitochondrial dysfunction, apoptosis and sarcopenia in skeletal muscle of mitochondrial DNA mutator mice. PLoS One (2010) 1.57
Mitochondrial stress engages E2F1 apoptotic signaling to cause deafness. Cell (2012) 1.56
Mitochondrial subversion in cancer. Cancer Prev Res (Phila) (2011) 1.54
Signaling pathways in mitochondrial dysfunction and aging. Mech Ageing Dev (2010) 1.54
Contribution of impaired mitochondrial autophagy to cardiac aging: mechanisms and therapeutic opportunities. Circ Res (2012) 1.53
Mitochondria as chi. Genetics (2008) 1.53
No variation and low synonymous substitution rates in coral mtDNA despite high nuclear variation. BMC Evol Biol (2006) 1.52
Stem cell aging: mechanisms, regulators and therapeutic opportunities. Nat Med (2014) 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
Current concepts in age-related hearing loss: epidemiology and mechanistic pathways. Hear Res (2013) 1.51
Epigenetics and its implications for behavioral neuroendocrinology. Front Neuroendocrinol (2008) 1.50
Aging perturbs 26S proteasome assembly in Drosophila melanogaster. FASEB J (2007) 1.49
Cardiac aging in mice and humans: the role of mitochondrial oxidative stress. Trends Cardiovasc Med (2009) 1.48
Mitochondrial reactive oxygen species. Contribution to oxidative stress and interorganellar signaling. Plant Physiol (2006) 1.47
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
Age-dependent cardiomyopathy in mitochondrial mutator mice is attenuated by overexpression of catalase targeted to mitochondria. Aging Cell (2010) 1.47
Caspase-2 deficiency enhances aging-related traits in mice. Mech Ageing Dev (2006) 1.46
The microbial metabolites, short-chain fatty acids, regulate colonic Treg cell homeostasis. Science (2013) 8.53
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
DNA replication and transcription in mammalian mitochondria. Annu Rev Biochem (2007) 3.74
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
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
Parkinsonism, premature menopause, and mitochondrial DNA polymerase gamma mutations: clinical and molecular genetic study. Lancet (2004) 2.91
Germline mitochondrial DNA mutations aggravate ageing and can impair brain development. Nature (2013) 2.90
Ablation of PGC-1beta results in defective mitochondrial activity, thermogenesis, hepatic function, and cardiac performance. PLoS Biol (2006) 2.86
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
Composition and dynamics of human mitochondrial nucleoids. Mol Biol Cell (2003) 2.74
The role of mitochondria in aging. J Clin Invest (2013) 2.71
What causes mitochondrial DNA deletions in human cells? Nat Genet (2008) 2.70
Reconstitution of a minimal mtDNA replisome in vitro. EMBO J (2004) 2.49
Mutations in amphiphysin 2 (BIN1) disrupt interaction with dynamin 2 and cause autosomal recessive centronuclear myopathy. Nat Genet (2007) 2.48
Increased mitochondrial mass in mitochondrial myopathy mice. Proc Natl Acad Sci U S A (2002) 2.38
Biased incorporation of ribonucleotides on the mitochondrial L-strand accounts for apparent strand-asymmetric DNA replication. Cell (2002) 2.32
TWINKLE Has 5' -> 3' DNA helicase activity and is specifically stimulated by mitochondrial single-stranded DNA-binding protein. J Biol Chem (2003) 2.24
Somatic progenitor cell vulnerability to mitochondrial DNA mutagenesis underlies progeroid phenotypes in Polg mutator mice. Cell Metab (2012) 2.21
Mutations in the phospholipid remodeling gene SERAC1 impair mitochondrial function and intracellular cholesterol trafficking and cause dystonia and deafness. Nat Genet (2012) 2.12
Contrast echocardiography reveals apparently normal coronary perfusion in a rat model of stress-induced (Takotsubo) cardiomyopathy. Eur Heart J Cardiovasc Imaging (2013) 2.10
Twinkle helicase is essential for mtDNA maintenance and regulates mtDNA copy number. Hum Mol Genet (2004) 2.00
Unraveling the structure of DNA during overstretching by using multicolor, single-molecule fluorescence imaging. Proc Natl Acad Sci U S A (2009) 1.95
Variation in germline mtDNA heteroplasmy is determined prenatally but modified during subsequent transmission. Nat Genet (2012) 1.94
Replication of vertebrate mitochondrial DNA entails transient ribonucleotide incorporation throughout the lagging strand. EMBO J (2006) 1.93
Hereditary myopathy with early respiratory failure associated with a mutation in A-band titin. Brain (2012) 1.92
Improved glucose control and reduced body fat mass in free fatty acid receptor 2-deficient mice fed a high-fat diet. Am J Physiol Endocrinol Metab (2010) 1.90
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
Mutant mitochondrial helicase Twinkle causes multiple mtDNA deletions and a late-onset mitochondrial disease in mice. Proc Natl Acad Sci U S A (2005) 1.88
Mammalian mitochondrial DNA replicates bidirectionally from an initiation zone. J Biol Chem (2003) 1.83
"Necklace" fibers, a new histological marker of late-onset MTM1-related centronuclear myopathy. Acta Neuropathol (2008) 1.83
Human Dna2 is a nuclear and mitochondrial DNA maintenance protein. Mol Cell Biol (2009) 1.83
No influence of Indy on lifespan in Drosophila after correction for genetic and cytoplasmic background effects. PLoS Genet (2007) 1.82
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
Response: The mitochondrial DNA replication bubble has not burst. Trends Biochem Sci (2003) 1.80
A bidirectional origin of replication maps to the major noncoding region of human mitochondrial DNA. Mol Cell (2005) 1.79
The human SIRT3 protein deacetylase is exclusively mitochondrial. Biochem J (2008) 1.74
Expression of catalytic mutants of the mtDNA helicase Twinkle and polymerase POLG causes distinct replication stalling phenotypes. Nucleic Acids Res (2007) 1.69
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
Twinkle helicase (PEO1) gene mutation causes mitochondrial DNA depletion. Ann Neurol (2007) 1.67
Prostate hyperplasia in a transgenic mouse with prostate-specific expression of prolactin. Endocrinology (2003) 1.63
Mitochondrial medicine: a metabolic perspective on the pathology of oxidative phosphorylation disorders. Cell Metab (2006) 1.62
Ghrelin increases intake of rewarding food in rodents. Addict Biol (2010) 1.61
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
Opposing effects of adiponectin receptors 1 and 2 on energy metabolism. Diabetes (2007) 1.59
Acutely reduced locomotor activity is a major contributor to Western diet-induced obesity in mice. Am J Physiol Endocrinol Metab (2007) 1.58
ETFDH mutations as a major cause of riboflavin-responsive multiple acyl-CoA dehydrogenation deficiency. Brain (2007) 1.55
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
The AAA+ protein ATAD3 has displacement loop binding properties and is involved in mitochondrial nucleoid organization. J Cell Biol (2007) 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
Hereditary myopathy with early respiratory failure: occurrence in various populations. J Neurol Neurosurg Psychiatry (2013) 1.52
Alterations to the expression level of mitochondrial transcription factor A, TFAM, modify the mode of mitochondrial DNA replication in cultured human cells. Nucleic Acids Res (2006) 1.51
Human mitochondrial transcription factor A induces a U-turn structure in the light strand promoter. Nat Struct Mol Biol (2011) 1.51
Mammalian mitochondrial DNA replication intermediates are essentially duplex but contain extensive tracts of RNA/DNA hybrid. J Mol Biol (2010) 1.50
Mammalian mitochondrial nucleoids: organizing an independently minded genome. Mitochondrion (2007) 1.49
Aberrant mitochondrial iron distribution and maturation arrest characterize early erythroid precursors in low-risk myelodysplastic syndromes. Blood (2005) 1.44
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
Hexosamine biosynthetic pathway mutations cause neuromuscular transmission defect. Am J Hum Genet (2011) 1.39
Infantile onset spinocerebellar ataxia is caused by recessive mutations in mitochondrial proteins Twinkle and Twinky. Hum Mol Genet (2005) 1.38
Twinkle and POLG defects enhance age-dependent accumulation of mutations in the control region of mtDNA. Nucleic Acids Res (2004) 1.38
The mitochondrial transcription termination factor mTERF modulates replication pausing in human mitochondrial DNA. Nucleic Acids Res (2007) 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