Published in Biochim Biophys Acta on December 08, 2011
After the banquet: mitochondrial biogenesis, mitophagy, and cell survival. Autophagy (2013) 1.17
Mitochondrial dynamics and mitochondrial quality control. Redox Biol (2014) 1.09
Exploring new pathways of neurodegeneration in ALS: the role of mitochondria quality control. Brain Res (2014) 0.84
The mitochondrial nucleoid: integrating mitochondrial DNA into cellular homeostasis. Cold Spring Harb Perspect Biol (2013) 0.84
Mitochondrial biogenesis-associated factors underlie the magnitude of response to aerobic endurance training in rats. Pflugers Arch (2014) 0.83
A Mechanistic Review of Mitophagy and Its Role in Protection against Alcoholic Liver Disease. Biomolecules (2015) 0.83
Mitochondrial cereblon functions as a Lon-type protease. Sci Rep (2016) 0.79
NOA1, a novel ClpXP substrate, takes an unexpected nuclear detour prior to mitochondrial import. PLoS One (2014) 0.78
Cross Talk of Proteostasis and Mitostasis in Cellular Homeodynamics, Ageing, and Disease. Oxid Med Cell Longev (2016) 0.77
Mitochondrial ClpP activity is required for cisplatin resistance in human cells. Biochim Biophys Acta (2015) 0.75
Energizing Genetics and Epi-genetics: Role in the Regulation of Mitochondrial Function. Curr Genomics (2014) 0.75
Tetramethylpyrazine blocks TFAM degradation and up-regulates mitochondrial DNA copy number by interacting with TFAM. Biosci Rep (2017) 0.75
Mitochondrial Quality Control Proteases in Neuronal Welfare. J Neuroimmune Pharmacol (2016) 0.75
Mitochondrial Biogenesis in Response to Chromium (VI) Toxicity in Human Liver Cells. Int J Mol Sci (2017) 0.75
Drosophila protease ClpXP specifically degrades DmLRPPRC1 controlling mitochondrial mRNA and translation. Sci Rep (2017) 0.75
Gene expression profile of aging and its retardation by caloric restriction. Science (1999) 8.06
Mitochondrial transcription factor A is necessary for mtDNA maintenance and embryogenesis in mice. Nat Genet (1998) 7.89
Transcriptional paradigms in mammalian mitochondrial biogenesis and function. Physiol Rev (2008) 6.57
Mitochondrial transcription factors B1 and B2 activate transcription of human mtDNA. Nat Genet (2002) 4.48
Spastic paraplegia and OXPHOS impairment caused by mutations in paraplegin, a nuclear-encoded mitochondrial metalloprotease. Cell (1998) 4.41
Similarity of human mitochondrial transcription factor 1 to high mobility group proteins. Science (1991) 4.26
Mitochondrial transcription factor A regulates mtDNA copy number in mammals. Hum Mol Genet (2004) 4.11
A close relative of the nuclear, chromosomal high-mobility group protein HMG1 in yeast mitochondria. Proc Natl Acad Sci U S A (1991) 3.37
Lon protease preferentially degrades oxidized mitochondrial aconitase by an ATP-stimulated mechanism. Nat Cell Biol (2002) 2.88
MTERF3 is a negative regulator of mammalian mtDNA transcription. Cell (2007) 2.76
The layered structure of human mitochondrial DNA nucleoids. J Biol Chem (2007) 2.74
Composition and dynamics of human mitochondrial nucleoids. Mol Biol Cell (2003) 2.74
Mutations in the mitochondrial protease gene AFG3L2 cause dominant hereditary ataxia SCA28. Nat Genet (2010) 2.66
The organization and inheritance of the mitochondrial genome. Nat Rev Genet (2005) 2.57
The mitochondrial transcription factor TFAM coordinates the assembly of multiple DNA molecules into nucleoid-like structures. Mol Biol Cell (2007) 2.42
Prohibitins regulate membrane protein degradation by the m-AAA protease in mitochondria. Mol Cell Biol (1999) 2.34
ClpXP, an ATP-powered unfolding and protein-degradation machine. Biochim Biophys Acta (2011) 2.17
Downregulation of the human Lon protease impairs mitochondrial structure and function and causes cell death. Free Radic Biol Med (2005) 2.06
Human mitochondrial DNA is packaged with TFAM. Nucleic Acids Res (2003) 2.01
Architectural role of mitochondrial transcription factor A in maintenance of human mitochondrial DNA. Mol Cell Biol (2004) 1.97
Functions of the high mobility group protein, Abf2p, in mitochondrial DNA segregation, recombination and copy number in Saccharomyces cerevisiae. Genetics (1998) 1.85
TFB2 is a transient component of the catalytic site of the human mitochondrial RNA polymerase. Cell (2009) 1.75
PIM1 encodes a mitochondrial ATP-dependent protease that is required for mitochondrial function in the yeast Saccharomyces cerevisiae. J Biol Chem (1994) 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
In vivo functional analysis of the human mitochondrial DNA polymerase POLG expressed in cultured human cells. J Biol Chem (2000) 1.68
Modulation of mitochondrial transcription in response to mtDNA depletion and repletion in HeLa cells. Nucleic Acids Res (2002) 1.64
Regulation of mitochondrial D-loops by transcription factor A and single-stranded DNA-binding protein. EMBO Rep (2002) 1.61
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
Quality control of mitochondrial proteostasis. Cold Spring Harb Perspect Biol (2011) 1.55
Prohibitin and mitochondrial biology. Trends Endocrinol Metab (2009) 1.54
The N-terminal zinc binding domain of ClpX is a dimerization domain that modulates the chaperone function. J Biol Chem (2003) 1.54
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
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
The mitochondrial transcription termination factor mTERF modulates replication pausing in human mitochondrial DNA. Nucleic Acids Res (2007) 1.38
Drosophila mitochondrial transcription factor B1 modulates mitochondrial translation but not transcription or DNA copy number in Schneider cells. J Biol Chem (2005) 1.37
An enzyme in yeast mitochondria that catalyzes a step in branched-chain amino acid biosynthesis also functions in mitochondrial DNA stability. EMBO J (1995) 1.37
Drosophila mitochondrial transcription factor B2 regulates mitochondrial DNA copy number and transcription in schneider cells. J Biol Chem (2004) 1.34
Core human mitochondrial transcription apparatus is a regulated two-component system in vitro. Proc Natl Acad Sci U S A (2010) 1.34
Roles for the human ATP-dependent Lon protease in mitochondrial DNA maintenance. J Biol Chem (2007) 1.34
Transmission of cell stress from endoplasmic reticulum to mitochondria: enhanced expression of Lon protease. J Cell Biol (2002) 1.33
Human prohibitin 1 maintains the organization and stability of the mitochondrial nucleoids. Exp Cell Res (2008) 1.28
Degrons in protein substrates program the speed and operating efficiency of the AAA+ Lon proteolytic machine. Proc Natl Acad Sci U S A (2009) 1.27
Drosophila mitochondrial transcription factor A (d-TFAM) is dispensable for the transcription of mitochondrial DNA in Kc167 cells. Biochem J (2001) 1.23
AAA proteases in mitochondria: diverse functions of membrane-bound proteolytic machines. Res Microbiol (2009) 1.22
Rearrangements of human mitochondrial DNA (mtDNA): new insights into the regulation of mtDNA copy number and gene expression. Mol Biol Cell (2000) 1.21
Structural analysis and DNA binding of the HMG domains of the human mitochondrial transcription factor A. Nucleic Acids Res (2009) 1.21
Turnover of mitochondrial steroidogenic acute regulatory (StAR) protein by Lon protease: the unexpected effect of proteasome inhibitors. Mol Endocrinol (2007) 1.20
Emerging roles of mitochondrial proteases in neurodegeneration. Biochim Biophys Acta (2009) 1.19
Oxidized mitochondrial protein degradation and repair in aging and oxidative stress. Antioxid Redox Signal (2010) 1.19
Mitochondrial Lon protease is a human stress protein. Free Radic Biol Med (2009) 1.18
Importance of the lon protease in mitochondrial maintenance and the significance of declining lon in aging. Ann N Y Acad Sci (2007) 1.17
Increasing organismal healthspan by enhancing mitochondrial protein quality control. Nat Cell Biol (2009) 1.17
High mitochondrial DNA copy number has detrimental effects in mice. Hum Mol Genet (2010) 1.15
DNA and RNA binding by the mitochondrial lon protease is regulated by nucleotide and protein substrate. J Biol Chem (2004) 1.13
Mitochondrial enzymes are protected from stress-induced aggregation by mitochondrial chaperones and the Pim1/LON protease. Mol Biol Cell (2011) 1.12
Proteomic analysis of mitochondrial protein turnover: identification of novel substrate proteins of the matrix protease pim1. Mol Cell Biol (2006) 1.12
Differential phenotypes of active site and human autosomal dominant progressive external ophthalmoplegia mutations in Drosophila mitochondrial DNA helicase expressed in Schneider cells. J Biol Chem (2007) 1.11
Mitochondrial Lon of Saccharomyces cerevisiae is a ring-shaped protease with seven flexible subunits. Proc Natl Acad Sci U S A (1999) 1.10
DNA stimulates ATP-dependent proteolysis and protein-dependent ATPase activity of protease La from Escherichia coli. Proc Natl Acad Sci U S A (1982) 1.09
MTERF2 is a nucleoid component in mammalian mitochondria. Biochim Biophys Acta (2009) 1.09
Functional mechanics of the ATP-dependent Lon protease- lessons from endogenous protein and synthetic peptide substrates. Biochim Biophys Acta (2008) 1.09
The ATP-dependent Lon protease of Mus musculus is a DNA-binding protein that is functionally conserved between yeast and mammals. Gene (2003) 1.08
The ATPase and protease domains of yeast mitochondrial Lon: roles in proteolysis and respiration-dependent growth. Proc Natl Acad Sci U S A (1998) 1.07
ATP-dependent proteolysis in mitochondria. m-AAA protease and PIM1 protease exert overlapping substrate specificities and cooperate with the mtHsp70 system. J Biol Chem (1998) 1.05
Functional domains of Brevibacillus thermoruber lon protease for oligomerization and DNA binding: role of N-terminal and sensor and substrate discrimination domains. J Biol Chem (2004) 1.01
The role of protein quality control in mitochondrial protein homeostasis under oxidative stress. Proteomics (2010) 1.00
Mitochondrial DNA transcription regulation and nucleoid organization. J Inherit Metab Dis (2011) 1.00
Changes in rat liver mitochondria with aging. Lon protease-like reactivity and N(epsilon)-carboxymethyllysine accumulation in the matrix. Eur J Biochem (2003) 1.00
Yeast aconitase binds and provides metabolically coupled protection to mitochondrial DNA. Proc Natl Acad Sci U S A (2007) 0.99
Requirement for the yeast gene LON in intramitochondrial proteolysis and maintenance of respiration. Science (1994) 0.99
Mutations in the proteolytic domain of Escherichia coli protease Lon impair the ATPase activity of the enzyme. FEBS Lett (1998) 0.96
Mitochondrial DNA instability mutants of the bifunctional protein Ilv5p have altered organization in mitochondria and are targeted for degradation by Hsp78 and the Pim1p protease. J Biol Chem (2002) 0.96
Functional domains of chicken mitochondrial transcription factor A for the maintenance of mitochondrial DNA copy number in lymphoma cell line DT40. J Biol Chem (2003) 0.96
The ATP-dependent PIM1 protease is required for the expression of intron-containing genes in mitochondria. Genes Dev (1998) 0.96
The human LON protease binds to mitochondrial promoters in a single-stranded, site-specific, strand-specific manner. Biochemistry (1998) 0.96
Identification of novel oxidized protein substrates and physiological partners of the mitochondrial ATP-dependent Lon-like protease Pim1. J Biol Chem (2010) 0.95
Effects of inorganic polyphosphate on the proteolytic and DNA-binding activities of Lon in Escherichia coli. J Biol Chem (2004) 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
DNA-stimulated ATPase activity on the lon (CapR) protein. J Bacteriol (1984) 0.93
Diverse functions of mitochondrial AAA+ proteins: protein activation, disaggregation, and degradation. Biochem Cell Biol (2010) 0.92
Overexpression of MTERFD1 or MTERFD3 impairs the completion of mitochondrial DNA replication. Mol Biol Rep (2010) 0.92
A point mutation within the ATP-binding site inactivates both catalytic functions of the ATP-dependent protease La (Lon) from Escherichia coli. FEBS Lett (1994) 0.91
A mechanistic view of human mitochondrial DNA polymerase gamma: providing insight into drug toxicity and mitochondrial disease. Biochim Biophys Acta (2010) 0.88
Structure of the catalytic domain of the human mitochondrial Lon protease: proposed relation of oligomer formation and activity. Protein Sci (2010) 0.88
Mcx1p, a ClpX homologue in mitochondria of Saccharomyces cerevisiae. FEBS Lett (1998) 0.88
Mitochondrial DNA nucleoids determine mitochondrial genetics and dysfunction. Int J Biochem Cell Biol (2009) 0.87
A Drosophila model of mitochondrial DNA replication: proteins, genes and regulation. IUBMB Life (2005) 0.87
The core human mitochondrial transcription initiation complex: It only takes two to tango. Transcription (2011) 0.87
Thermodynamic characterization of specific interactions between the human Lon protease and G-quartet DNA. Nucleic Acids Res (2008) 0.86
Transcriptional and cellular responses to defective mitochondrial proteolysis in fission yeast. J Mol Biol (2011) 0.84
The Drosophila nuclear factor DREF positively regulates the expression of the mitochondrial transcription termination factor DmTTF. Biochem J (2009) 0.84
Assembly factors and ATP-dependent proteases in cytochrome c oxidase biogenesis. Biochim Biophys Acta (2010) 0.84
Mitochondrial chromosome structure: an insight from analysis of complete yeast genomes. FEMS Yeast Res (2006) 0.83
DNA-binding specificity of the Lon protease alpha-domain from Brevibacillus thermoruber WR-249. Biochem Biophys Res Commun (2009) 0.81
Maintenance and stabilization of mtDNA can be facilitated by the DNA-binding activity of Ilv5p. Biochim Biophys Acta (2007) 0.81
Mitochondrial transcription: how does it end? Transcription (2012) 0.80
Transcriptional regulation of the Drosophila ANT gene by the DRE/DREF system. Genes Cells (2007) 0.79
Protein substrates and heat shock reduce the DNA-binding ability of Escherichia coli Lon protease. Appl Microbiol Biotechnol (1995) 0.79
Differential degradation of variant medium-chain acyl-CoA dehydrogenase by the protein quality control proteases Lon and ClpXP. Biochem Biophys Res Commun (2005) 0.77
Functional defects due to spacer-region mutations of human mitochondrial DNA polymerase in a family with an ataxia-myopathy syndrome. Hum Mol Genet (2005) 1.58
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
Drosophila mitochondrial transcription factor B1 modulates mitochondrial translation but not transcription or DNA copy number in Schneider cells. J Biol Chem (2005) 1.37
Drosophila mitochondrial transcription factor B2 regulates mitochondrial DNA copy number and transcription in schneider cells. J Biol Chem (2004) 1.34
A novel processive mechanism for DNA synthesis revealed by structure, modeling and mutagenesis of the accessory subunit of human mitochondrial DNA polymerase. J Mol Biol (2006) 1.25
Modular architecture of the hexameric human mitochondrial DNA helicase. J Mol Biol (2007) 1.21
Differential phenotypes of active site and human autosomal dominant progressive external ophthalmoplegia mutations in Drosophila mitochondrial DNA helicase expressed in Schneider cells. J Biol Chem (2007) 1.11
Physiological and biochemical defects in functional interactions of mitochondrial DNA polymerase and DNA-binding mutants of single-stranded DNA-binding protein. J Biol Chem (2004) 1.09
Clustering of Alpers disease mutations and catalytic defects in biochemical variants reveal new features of molecular mechanism of the human mitochondrial replicase, Pol γ. Nucleic Acids Res (2011) 1.08
The accessory subunit of DNA polymerase gamma is essential for mitochondrial DNA maintenance and development in Drosophila melanogaster. Proc Natl Acad Sci U S A (2002) 1.05
Mutations in the spacer region of Drosophila mitochondrial DNA polymerase affect DNA binding, processivity, and the balance between Pol and Exo function. J Biol Chem (2004) 1.03
Functional roles of the N- and C-terminal regions of the human mitochondrial single-stranded DNA-binding protein. PLoS One (2010) 1.00
Physiological and biochemical defects in carboxyl-terminal mutants of mitochondrial DNA helicase. J Biol Chem (2008) 0.98
Mg2+-assisted catalysis by B. stearothermophilus TrpRS is promoted by allosteric effects. Structure (2009) 0.96
Dynamic effects of cofactors and DNA on the oligomeric state of human mitochondrial DNA helicase. J Biol Chem (2010) 0.93
Over-expression of the catalytic core of mitochondrial DNA (mtDNA) polymerase in the nervous system of Drosophila melanogaster reduces median life span by inducing mtDNA depletion. J Neurochem (2007) 0.93
Animal models of mitochondrial DNA transactions in disease and ageing. Exp Gerontol (2010) 0.93
Mitochondrial transcription factor B2 is essential for metabolic function in Drosophila melanogaster development. J Biol Chem (2008) 0.91
Drosophila melanogaster as a model system to study mitochondrial biology. Methods Mol Biol (2007) 0.90
A cytoplasmic suppressor of a nuclear mutation affecting mitochondrial functions in Drosophila. Genetics (2012) 0.89
Functional importance of the conserved N-terminal domain of the mitochondrial replicative DNA helicase. Biochim Biophys Acta (2008) 0.89
Comparative purification strategies for Drosophila and human mitochondrial DNA replication proteins: DNA polymerase gamma and mitochondrial single-stranded DNA-binding protein. Methods Mol Biol (2009) 0.88
A Drosophila model of mitochondrial DNA replication: proteins, genes and regulation. IUBMB Life (2005) 0.87
Disruption of mitochondrial DNA replication in Drosophila increases mitochondrial fast axonal transport in vivo. PLoS One (2009) 0.87
Modeling pathogenic mutations of human twinkle in Drosophila suggests an apoptosis role in response to mitochondrial defects. PLoS One (2012) 0.86
Parallel multiplicative target screening against divergent bacterial replicases: identification of specific inhibitors with broad spectrum potential. Biochemistry (2010) 0.86
Modeling human mitochondrial diseases in flies. Biochim Biophys Acta (2006) 0.85
Functional analysis of H. sapiens DNA polymerase gamma spacer mutation W748S with and without common variant E1143G. Biochim Biophys Acta (2010) 0.81
Coiled coil domain-containing protein 56 (CCDC56) is a novel mitochondrial protein essential for cytochrome c oxidase function. J Biol Chem (2012) 0.81
Purification strategy for recombinant forms of the human mitochondrial DNA helicase. Methods Mol Biol (2009) 0.80
Purification strategies for Drosophila mtDNA replication proteins in native and recombinant form. Mitochondrial single-stranded DNA-binding protein. Methods Mol Biol (2002) 0.79
Purification strategies for Drosophila mtDNA replication proteins in native and recombinant form. DNA polymerase gamma. Methods Mol Biol (2002) 0.78
Functional analysis by inducible RNA interference in Drosophila melanogaster. Methods Mol Biol (2007) 0.78
Drosophila nuclear factor DREF regulates the expression of the mitochondrial DNA helicase and mitochondrial transcription factor B2 but not the mitochondrial translation factor B1. Biochim Biophys Acta (2013) 0.78
Physical analysis of recombinant forms of the human mitochondrial DNA helicase. Methods (2010) 0.76
Muscle-specific overexpression of the catalytic subunit of DNA polymerase γ induces pupal lethality in Drosophila melanogaster. Arch Insect Biochem Physiol (2013) 0.75
Co-occurrence of four nucleotide changes associated with an adult mitochondrial ataxia phenotype. BMC Res Notes (2014) 0.75
Iron-Sulfur Clusters in Mitochondrial Metabolism: Multifaceted Roles of a Simple Cofactor. Biochemistry (Mosc) (2016) 0.75