Published in Hum Mol Genet on March 09, 2005
Mitochondrial DNA mutations in disease and aging. J Cell Biol (2011) 1.67
Ca2+-calmodulin-dependent protein kinase II represses cardiac transcription of the L-type calcium channel alpha(1C)-subunit gene (Cacna1c) by DREAM translocation. J Physiol (2011) 1.16
Regulation of excitation-contraction coupling in mouse cardiac myocytes: integrative analysis with mathematical modelling. BMC Physiol (2009) 0.91
Mouse models for nuclear DNA-encoded mitochondrial complex I deficiency. J Inherit Metab Dis (2010) 0.90
Cysteinyl leukotriene signaling through perinuclear CysLT(1) receptors on vascular smooth muscle cells transduces nuclear calcium signaling and alterations of gene expression. J Mol Med (Berl) (2012) 0.79
Calcium signalling in developing cardiomyocytes: implications for model systems and disease. J Physiol (2015) 0.78
Metabolic manipulators: a well founded strategy to combat mitochondrial dysfunction. J Inherit Metab Dis (2010) 0.76
Peroxisome proliferator-activated receptor-γ coactivator 1 α1 induces a cardiac excitation-contraction coupling phenotype without metabolic remodelling. J Physiol (2016) 0.75
Vascular Endothelial Growth Factor-B Induces a Distinct Electrophysiological Phenotype in Mouse Heart. Front Physiol (2017) 0.75
Mitochondrial cardiomyopathies feature increased uptake and diminished efflux of mitochondrial calcium. J Mol Cell Cardiol (2017) 0.75
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
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
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
Somatic progenitor cell vulnerability to mitochondrial DNA mutagenesis underlies progeroid phenotypes in Polg mutator mice. Cell Metab (2012) 2.21
Variation in germline mtDNA heteroplasmy is determined prenatally but modified during subsequent transmission. Nat Genet (2012) 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
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
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
Respiratory and limb muscle weakness induced by tumor necrosis factor-alpha: involvement of muscle myofilaments. Am J Respir Crit Care Med (2002) 1.72
MTERF4 regulates translation by targeting the methyltransferase NSUN4 to the mammalian mitochondrial ribosome. Cell Metab (2011) 1.68
Mitochondrial DNA mutations in disease and aging. J Cell Biol (2011) 1.67
Cross bridges account for only 20% of total ATP consumption during submaximal isometric contraction in mouse fast-twitch skeletal muscle. Am J Physiol Cell Physiol (2006) 1.62
Ultra-deep sequencing of mouse mitochondrial DNA: mutational patterns and their origins. PLoS Genet (2011) 1.60
Purifying selection of mtDNA and its implications for understanding evolution and mitochondrial disease. Nat Rev Genet (2008) 1.60
Effects of congestive heart failure on Ca2+ handling in skeletal muscle during fatigue. Circ Res (2006) 1.57
Role of reactive oxygen species in contraction-mediated glucose transport in mouse skeletal muscle. J Physiol (2006) 1.55
A switch in metabolism precedes increased mitochondrial biogenesis in respiratory chain-deficient mouse hearts. Proc Natl Acad Sci U S A (2004) 1.53
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
Muscle glycogen stores and fatigue. J Physiol (2013) 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
Mammalian transcription factor A is a core component of the mitochondrial transcription machinery. Proc Natl Acad Sci U S A (2012) 1.37
Hypermetabolism in mice caused by the central action of an unliganded thyroid hormone receptor alpha1. EMBO J (2007) 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
Effects of palmitate on Ca(2+) handling in adult control and ob/ob cardiomyocytes: impact of mitochondrial reactive oxygen species. Diabetes (2007) 1.31
Increased mitochondrial Ca2+ and decreased sarcoplasmic reticulum Ca2+ in mitochondrial myopathy. Hum Mol Genet (2008) 1.29
Acute effects of reactive oxygen and nitrogen species on the contractile function of skeletal muscle. J Physiol (2010) 1.28
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
Limited oxygen diffusion accelerates fatigue development in mouse skeletal muscle. J Physiol (2006) 1.25
Impaired nigrostriatal function precedes behavioral deficits in a genetic mitochondrial model of Parkinson's disease. FASEB J (2011) 1.19
Dietary nitrate increases tetanic [Ca2+]i and contractile force in mouse fast-twitch muscle. J Physiol (2012) 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
Mitochondrial and myoplasmic [Ca2+] in single fibres from mouse limb muscles during repeated tetanic contractions. J Physiol (2003) 1.18
Doxorubicin acts through tumor necrosis factor receptor subtype 1 to cause dysfunction of murine skeletal muscle. J Appl Physiol (1985) (2009) 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
Mitochondrial production of reactive oxygen species contributes to the β-adrenergic stimulation of mouse cardiomycytes. J Physiol (2011) 1.13
Muscle fatigue: from observations in humans to underlying mechanisms studied in intact single muscle fibres. Eur J Appl Physiol (2010) 1.13
Pacing-induced calcineurin activation controls cardiac Ca2+ signalling and gene expression. J Physiol (2003) 1.12
Increased fatigue resistance linked to Ca2+-stimulated mitochondrial biogenesis in muscle fibres of cold-acclimated mice. J Physiol (2010) 1.10
The bicoid stability factor controls polyadenylation and expression of specific mitochondrial mRNAs in Drosophila melanogaster. PLoS 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
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
Ryanodine receptors of pancreatic beta-cells mediate a distinct context-dependent signal for insulin secretion. FASEB J (2002) 1.05
Effects of HMGB1 on in vitro responses of isolated muscle fibers and functional aspects in skeletal muscles of idiopathic inflammatory myopathies. FASEB J (2009) 1.04
Difference in skeletal muscle function in males vs. females: role of estrogen receptor-beta. Am J Physiol Endocrinol Metab (2004) 1.04
Antioxidant treatments do not improve force recovery after fatiguing stimulation of mouse skeletal muscle fibres. J Physiol (2014) 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
The role of Ca2+ influx for insulin-mediated glucose uptake in skeletal muscle. Diabetes (2006) 1.01
Local arginase inhibition during early reperfusion mediates cardioprotection via increased nitric oxide production. PLoS One (2012) 1.01
Loss of LRPPRC causes ATP synthase deficiency. Hum Mol Genet (2014) 1.01
Effects of glucose on contractile function, [Ca2+]i, and glycogen in isolated mouse skeletal muscle. Am J Physiol Cell Physiol (2002) 1.00
Locomotor deficiencies and aberrant development of subtype-specific GABAergic interneurons caused by an unliganded thyroid hormone receptor alpha1. J Neurosci (2008) 1.00
Mitochondrial dysfunction in mammalian ageing. Novartis Found Symp (2007) 1.00
The role of in vivo Ca²⁺ signals acting on Ca²⁺-calmodulin-dependent proteins for skeletal muscle plasticity. J Physiol (2011) 0.99
Sequence-specific stalling of DNA polymerase γ and the effects of mutations causing progressive ophthalmoplegia. Hum Mol Genet (2011) 0.97
Somatic mtDNA mutations cause progressive hearing loss in the mouse. Exp Cell Res (2007) 0.97
Understanding aging: revealing order out of chaos. Biochim Biophys Acta (2004) 0.97
Secondary metabolic effects in complex I deficiency. Ann Neurol (2005) 0.97
Interpolated twitches in fatiguing single mouse muscle fibres: implications for the assessment of central fatigue. J Physiol (2008) 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
Calmodulin kinase modulates Ca2+ release in mouse skeletal muscle. J Physiol (2003) 0.96
Maintenance of respiratory chain function in mouse hearts with severely impaired mtDNA transcription. Nucleic Acids Res (2010) 0.96
Upregulation of MHC class I in transgenic mice results in reduced force-generating capacity in slow-twitch muscle. Muscle Nerve (2009) 0.96
Nonshivering thermogenesis protects against defective calcium handling in muscle. FASEB J (2008) 0.95