Mitochondrial DNA transcription and diseases: past, present and future.

[1]  D. Wallace,et al.  Functional estrogen receptors in the mitochondria of breast cancer cells. , 2006, Molecular biology of the cell.

[2]  A. Psarra,et al.  The mitochondrion as a primary site of action of steroid and thyroid hormones: Presence and action of steroid and thyroid hormone receptors in mitochondria of animal cells , 2006, Molecular and Cellular Endocrinology.

[3]  L. Levinger,et al.  Naturally Occurring Mutations in Human Mitochondrial Pre-tRNASer(UCN) Can Affect the Transfer Ribonuclease Z Cleavage Site, Processing Kinetics, and Substrate Secondary Structure* , 2006, Journal of Biological Chemistry.

[4]  L. Hurst,et al.  Hearing silence: non-neutral evolution at synonymous sites in mammals , 2006, Nature Reviews Genetics.

[5]  Laurent Excoffier,et al.  Conserved noncoding sequences are selectively constrained and not mutation cold spots , 2006, Nature Genetics.

[6]  T. Shutt,et al.  Bacteriophage origins of mitochondrial replication and transcription proteins. , 2006, Trends in genetics : TIG.

[7]  J. Griffith,et al.  Termination Factor-Mediated DNA Loop between Termination and Initiation Sites Drives Mitochondrial rRNA Synthesis , 2005, Cell.

[8]  C. Kang,et al.  DNA light-strand preferential recognition of human mitochondria transcription termination factor mTERF. , 2005, Journal of biochemistry and molecular biology.

[9]  R. Wiesner,et al.  A new mechanism for mtDNA pathogenesis: impairment of post-transcriptional maturation leads to severe depletion of mitochondrial tRNASer(UCN) caused by T7512C and G7497A point mutations , 2005, Nucleic acids research.

[10]  D. Geiger,et al.  Polyadenylation and Degradation of Human Mitochondrial RNA: the Prokaryotic Past Leaves Its Mark , 2005, Molecular and Cellular Biology.

[11]  C. Gustafsson,et al.  The Human Mitochondrial Transcription Termination Factor (mTERF) Is Fully Active in Vitro in the Non-phosphorylated Form* , 2005, Journal of Biological Chemistry.

[12]  H. Jacobs,et al.  A bidirectional origin of replication maps to the major noncoding region of human mitochondrial DNA. , 2005, Molecular cell.

[13]  T. Katoh,et al.  Human Mitochondrial mRNAs Are Stabilized with Polyadenylation Regulated by Mitochondria-specific Poly(A) Polymerase and Polynucleotide Phosphorylase* , 2005, Journal of Biological Chemistry.

[14]  F. Martínez-Azorín.  The Mitochondrial Ribomotor Hypothesis , 2005, IUBMB life.

[15]  J. Montoya,et al.  New DNA-binding activity of rat mitochondrial transcription termination factor (mTERF). , 2004, Journal of biochemistry.

[16]  H. Jacobs,et al.  The 7472insC mtDNA mutation impairs 5' and 3' processing of tRNA(Ser(UCN)). , 2004, Biochemical and biophysical research communications.

[17]  M. Beal,et al.  Alzheimer's brains harbor somatic mtDNA control-region mutations that suppress mitochondrial transcription and replication. , 2004, Proceedings of the National Academy of Sciences of the United States of America.

[18]  D. Ebert,et al.  Covariation of Mitochondrial Genome Size with Gene Lengths: Evidence for Gene Length Reduction During Mitochondrial Evolution , 2004, Journal of Molecular Evolution.

[19]  R. Lightowlers,et al.  Messenger RNA stability in mitochondria: different means to an end. , 2004, Trends in genetics : TIG.

[20]  G. Shadel.  A dual-function mitochondrial transcription factor tunes out deafness. , 2004, Molecular genetics and metabolism.

[21]  X. Estivill,et al.  Human mitochondrial transcription factor B1 as a modifier gene for hearing loss associated with the mitochondrial A1555G mutation. , 2004, Molecular genetics and metabolism.

[22]  M. Helm,et al.  A Monomer-to-Trimer Transition of the Human Mitochondrial Transcription Termination Factor (mTERF) Is Associated with a Loss of in Vitro Activity* , 2004, Journal of Biological Chemistry.

[23]  J. Montoya,et al.  Phosphorylation of rat mitochondrial transcription termination factor (mTERF) is required for transcription termination but not for binding to DNA. , 2004, Nucleic acids research.

[24]  H. Jacobs,et al.  Mammalian Mitochondrial DNA Replicates Bidirectionally from an Initiation Zone* , 2003, Journal of Biological Chemistry.

[25]  N. Bresolin,et al.  High mutational burden in the mtDNA control region from aged muscles: a single-fiber study , 2003, Neurobiology of Aging.

[26]  S. Seneca,et al.  Investigation of a pathogenic mtDNA microdeletion reveals a translation-dependent deadenylation decay pathway in human mitochondria. , 2003, Human molecular genetics.

[27]  R. Wiesner,et al.  Import of mitochondrial transcription factor A (TFAM) into rat liver mitochondria stimulates transcription of mitochondrial DNA. , 2003, Nucleic acids research.

[28]  G. Shadel,et al.  Human Mitochondrial Transcription Factor B1 Interacts with the C-Terminal Activation Region of h-mtTFA and Stimulates Transcription Independently of Its RNA Methyltransferase Activity , 2003, Molecular and Cellular Biology.

[29]  S. Mili,et al.  LRP130, a Pentatricopeptide Motif Protein with a Noncanonical RNA-Binding Domain, Is Bound In Vivo to Mitochondrial and Nuclear RNAs , 2003, Molecular and Cellular Biology.

[30]  M. Nashimoto,et al.  A candidate prostate cancer susceptibility gene encodes tRNA 3' processing endoribonuclease. , 2003, Nucleic acids research.

[31]  C. Sekeris,et al.  Oligonucleotide sequences similar to transcription factor consensi of nuclear genes are present in the human mitochondrial genome. , 2003, Anticancer research.

[32]  D. Wallace,et al.  Control region mtDNA variants: Longevity, climatic adaptation, and a forensic conundrum , 2003, Proceedings of the National Academy of Sciences of the United States of America.

[33]  N. Saitou,et al.  Synonymous mutations in the human dopamine receptor D2 (DRD2) affect mRNA stability and synthesis of the receptor. , 2003, Human molecular genetics.

[34]  Eric S. Lander,et al.  Identification of a gene causing human cytochrome c oxidase deficiency by integrative genomics , 2003, Proceedings of the National Academy of Sciences of the United States of America.

[35]  J. Enríquez,et al.  Replication and Transcription of Mammalian Mitochondrial Dna , 2003, Experimental physiology.

[36]  C. Gustafsson,et al.  Mitochondrial transcription factors B1 and B2 activate transcription of human mtDNA , 2002, Nature Genetics.

[37]  T. W. O'brien.  Evolution of a protein-rich mitochondrial ribosome: implications for human genetic disease. , 2002, Gene.

[38]  G. Shadel,et al.  A Human Mitochondrial Transcription Factor Is Related to RNA Adenine Methyltransferases and Binds S-Adenosylmethionine , 2002, Molecular and Cellular Biology.

[39]  M. James,et al.  In vitro 3'-end endonucleolytic processing defect in a human mitochondrial tRNA(Ser(UCN)) precursor with the U7445C substitution, which causes non-syndromic deafness. , 2001, Nucleic acids research.

[40]  Tsutomu Suzuki,et al.  Identification and Characterization of Mammalian Mitochondrial tRNA nucleotidyltransferases* , 2001, The Journal of Biological Chemistry.

[41]  S. Gensler,et al.  Mechanism of mammalian mitochondrial DNA replication: import of mitochondrial transcription factor A into isolated mitochondria stimulates 7S DNA synthesis. , 2001, Nucleic acids research.

[42]  J. Montoya,et al.  A study on the human mitochondrial RNA polymerase activity points to existence of a transcription factor B‐like protein , 2001, FEBS letters.

[43]  F. Casas,et al.  Thyroid hormone action in mitochondria. , 2001, Journal of molecular endocrinology.

[44]  G. Attardi,et al.  The RNase P Associated with HeLa Cell Mitochondria Contains an Essential RNA Component Identical in Sequence to That of the Nuclear RNase P , 2001, Molecular and Cellular Biology.

[45]  R Giegé,et al.  Search for characteristic structural features of mammalian mitochondrial tRNAs. , 2000, RNA.

[46]  K. Burnstein,et al.  Multiple androgen response elements and a Myc consensus site in the androgen receptor (AR) coding region are involved in androgen-mediated up-regulation of AR messenger RNA. , 1999, Molecular endocrinology.

[47]  N. Bresolin,et al.  Aging-dependent large accumulation of point mutations in the human mtDNA control region for replication. , 1999, Science.

[48]  D. Turnbull,et al.  Reanalysis and revision of the Cambridge reference sequence for human mitochondrial DNA , 1999, Nature Genetics.

[49]  J. Enríquez,et al.  Autonomous Regulation in Mammalian Mitochondrial DNA Transcription , 1999, Biological chemistry.

[50]  J. Enríquez,et al.  Direct Regulation of Mitochondrial RNA Synthesis by Thyroid Hormone , 1999, Molecular and Cellular Biology.

[51]  J. Montoya,et al.  Reduced mitochondrial DNA transcription in senescent rat heart. , 1998, Biochemical and biophysical research communications.

[52]  J. Enríquez,et al.  The Deafness-Associated Mitochondrial DNA Mutation at Position 7445, Which Affects tRNASer(UCN) Precursor Processing, Has Long-Range Effects on NADH Dehydrogenase Subunit ND6 Gene Expression , 1998, Molecular and Cellular Biology.

[53]  G. Attardi,et al.  The mtDNA‐encoded ND6 subunit of mitochondrial NADH dehydrogenase is essential for the assembly of the membrane arm and the respiratory function of the enzyme , 1998, The EMBO journal.

[54]  P. Moore,et al.  The structure of a methylated tetraloop in 16S ribosomal RNA. , 1998, Structure.

[55]  G. Barsh,et al.  Mitochondrial transcription factor A is necessary for mtDNA maintance and embryogenesis in mice , 1998, Nature Genetics.

[56]  C Saccone,et al.  Mammalian mitochondrial D-loop region structural analysis: identification of new conserved sequences and their functional and evolutionary implications. , 1997, Gene.

[57]  R. Wiesner,et al.  Regulation of mitochondrial transcription by mitochondrial transcription factor A , 1997, Molecular and Cellular Biochemistry.

[58]  M. Rocchi,et al.  Identification of the gene encoding the human mitochondrial RNA polymerase (h-mtRPOL) by cyberscreening of the Expressed Sequence Tags database. , 1997, Human molecular genetics.

[59]  P. Fernández-Silva,et al.  The human mitochondrial transcription termination factor (mTERF) is a multizipper protein but binds to DNA as a monomer, with evidence pointing to intramolecular leucine zipper interactions , 1997, The EMBO journal.

[60]  C. Overly,et al.  Organelle motility and metabolism in axons vs dendrites of cultured hippocampal neurons. , 1996, Journal of cell science.

[61]  C. Moraes,et al.  Functional and Molecular Mitochondrial Abnormalities Associated with a C T Transition at Position 3256 of the Human Mitochondrial Genome , 1996, The Journal of Biological Chemistry.

[62]  C. Sekeris,et al.  The mitochondrion as a primary site of action of glucocorticoids: the interaction of the glucocorticoid receptor with mitochondrial DNA sequences showing partial similarity to the nuclear glucocorticoid responsive elements , 1995, The Journal of Steroid Biochemistry and Molecular Biology.

[63]  I. Cassar-Malek,et al.  A 43-kDa Protein Related to c-Erb A α1 Is Located in the Mitochondrial Matrix of Rat Liver (*) , 1995, The Journal of Biological Chemistry.

[64]  Apollonia Tullo,et al.  Human Mitochondrial tRNA Processing (*) , 1995, The Journal of Biological Chemistry.

[65]  W. Hauswirth,et al.  In organello footprint analysis of human mitochondrial DNA: human mitochondrial transcription factor A interactions at the origin of replication , 1994, Molecular and cellular biology.

[66]  D. A. Clayton,et al.  Human mitochondrial transcription termination exhibits RNA polymerase independence and biased bipolarity in vitro. , 1994, The Journal of biological chemistry.

[67]  C. Saccone,et al.  Transcription of rat mitochondrial NADH‐dehydrogenase subunits presence of antisense and precursor RNA species , 1994, FEBS letters.

[68]  R. Wiesner,et al.  Coordination of nuclear and mitochondrial gene expression during the development of cardiac hypertrophy in rats. , 1994, The American journal of physiology.

[69]  F. Villarroya,et al.  Effects of cold environment on mitochondrial genome expression in the rat: evidence for a tissue-specific increase in the liver, independent of changes in mitochondrial gene abundance. , 1993, The Biochemical journal.

[70]  D. Turnbull,et al.  Abnormal RNA processing associated with a novel tRNA mutation in mitochondrial DNA. A potential disease mechanism. , 1993, The Journal of biological chemistry.

[71]  M. Wong-Riley,et al.  Mitochondrial and nuclear gene expression for cytochrome oxidase subunits are disproportionately regulated by functional activity in neurons , 1993, The Journal of neuroscience : the official journal of the Society for Neuroscience.

[72]  R. Aebersold,et al.  Molecular characterization of the transcription termination factor from human mitochondria. , 1993, The Journal of biological chemistry.

[73]  E. Schon,et al.  Fine mapping of mitochondrial RNAs derived from the mtDNA region containing a point mutation associated with MELAS. , 1993, Nucleic acids research.

[74]  R. Wiesner,et al.  Regulation by thyroid hormone of nuclear and mitochondrial genes encoding subunits of cytochrome-c oxidase in rat liver and skeletal muscle. , 1992, Molecular endocrinology.

[75]  I. Nonaka,et al.  MELAS mutation in mtDNA binding site for transcription termination factor causes defects in protein synthesis and in respiration but no change in levels of upstream and downstream mature transcripts. , 1992, Proceedings of the National Academy of Sciences of the United States of America.

[76]  M. Parisi,et al.  DNA wrapping and bending by a mitochondrial high mobility group-like transcriptional activator protein. , 1992, The Journal of biological chemistry.

[77]  E. Schon,et al.  Defects in mitochondrial protein synthesis and respiratory chain activity segregate with the tRNA(Leu(UUR)) mutation associated with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes. , 1992, Molecular and cellular biology.

[78]  P. Polosa,et al.  Distinctive pattern and translational control of mitochondrial protein synthesis in rat brain synaptic endings. , 1991, The Journal of biological chemistry.

[79]  D. A. Clayton,et al.  Similarity of human mitochondrial transcription factor 1 to high mobility group proteins. , 1991, Science.

[80]  M. Parisi,et al.  Impairment of mitochondrial transcription termination by a point mutation associated with the MELAS subgroup of mitochondrial encephalomyopathies , 1991, Nature.

[81]  V. Petruzzella,et al.  Reduced synthesis of mtRNA in isolated mitochondria of senescent rat brain. , 1991, Biochemical and biophysical research communications.

[82]  V. Petruzzella,et al.  Acetyl‐L‐carnitine increases cytochrome oxidase subunit I mRNA content in hypothyroid rat liver , 1990, FEBS letters.

[83]  C. M. V. Itallie,et al.  Thyroid hormone and dexamethasone increase the levels of a messenger ribonucleic acid for a mitochondrially encoded subunit but not for a nuclear-encoded subunit of cytochrome c oxidase. , 1990 .

[84]  E. Sbisà,et al.  The complete and symmetric transcription of the main non coding region of rat mitochondrial genome: in vivo mapping of heavy and light transcripts , 1990, Current Genetics.

[85]  V. Petruzzella,et al.  Reduced transcription of mitochondrial DNA in the senescent rat. Tissue dependence and effect of L-carnitine. , 1990, European journal of biochemistry.

[86]  G. Attardi,et al.  Termination of transcription in human mitochondria: Identification and purification of a DNA binding protein factor that promotes termination , 1989, Cell.

[87]  G. Michaels,et al.  Mechanism of mRNA binding to bovine mitochondrial ribosomes. , 1989, The Journal of biological chemistry.

[88]  Š. Kǔzela,et al.  Control of mitochondrial transcription by thyroid hormone. , 1989, European journal of biochemistry.

[89]  D. Pette,et al.  Chronic stimulation of rat skeletal muscle induces coordinate increases in mitochondrial and nuclear mRNAs of cytochrome-c-oxidase subunits. , 1989, European journal of biochemistry.

[90]  D. A. Clayton,et al.  Purification and characterization of human mitochondrial transcription factor 1 , 1988, Molecular and cellular biology.

[91]  J. N. Topper,et al.  Promoter selection in human mitochondria involves binding of a transcription factor to orientation-independent upstream regulatory elements , 1987, Cell.

[92]  G. Attardi,et al.  Markedly different ATP requirements for rRNA synthesis and mtDNA light strand transcription versus mRNA synthesis in isolated human mitochondria. , 1987, The Journal of biological chemistry.

[93]  R. S. Williams,et al.  Mitochondrial gene expression in mammalian striated muscle. Evidence that variation in gene dosage is the major regulatory event. , 1986, The Journal of biological chemistry.

[94]  P. Polosa,et al.  Quantitation of mitochondrial RNA species during rat liver development: the concentration of cytochrome oxidase subunit I (CoI) mRNA increases at birth. , 1986, Cell differentiation.

[95]  J. Walker,et al.  Two overlapping genes in bovine mitochondrial DNA encode membrane components of ATP synthase. , 1986, The EMBO journal.

[96]  R. Fisher,et al.  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. , 1985, The Journal of biological chemistry.

[97]  S. Altman,et al.  Characterization of an RNase P activity from HeLa cell mitochondria. Comparison with the cytosol RNase P activity. , 1985, The Journal of biological chemistry.

[98]  D. Bogenhagen,et al.  Identification of a promoter for transcription of the heavy strand of human mtDNA: In vitro transcription and deletion mutagenesis , 1984, Cell.

[99]  R. Doolittle,et al.  Identification of the polypeptides encoded in the ATPase 6 gene and in the unassigned reading frames 1 and 3 of human mtDNA. , 1983, Proceedings of the National Academy of Sciences of the United States of America.

[100]  J. Montoya,et al.  The pattern of transcription of the human mitochondrial rRNA genes reveals two overlapping transcription units , 1983, Cell.

[101]  M. Rabinowitz,et al.  Identification of initiation sites for heavy-strand and light-strand transcription in human mitochondrial DNA. , 1982, Proceedings of the National Academy of Sciences of the United States of America.

[102]  J. Montoya,et al.  Sequence analysis and precise mapping of the 3' ends of HeLa cell mitochondrial ribosomal RNAs. , 1982, Journal of molecular biology.

[103]  F. Sanger,et al.  Complete sequence of bovine mitochondrial DNA. Conserved features of the mammalian mitochondrial genome. , 1982, Journal of molecular biology.

[104]  F. Sanger,et al.  Sequence and organization of the human mitochondrial genome , 1981, Nature.

[105]  Julio Montoya,et al.  tRNA punctuation model of RNA processing in human mitochondria , 1981, Nature.

[106]  J. Montoya,et al.  Distinctive features of the 5′-terminal sequences of the human mitochondrial mRNAs , 1981, Nature.

[107]  T. P. Stewart,et al.  ‘Lipidic particles’ are intermembrane attachment sites , 1981, Nature.

[108]  F. Amalric,et al.  Fractionation of mitochondrial RNA from HeLa cells by high-resolution electrophoresis under strongly denaturing conditions. , 1978, Journal of molecular biology.

[109]  G. Attardi,et al.  Identification and partial characterization of multiple discrete polyadenylic acid containing RNA components coded for by HeLa cell mitochondrial DNA. , 1974, Journal of molecular biology.

[110]  S. Penman,et al.  Post-transcriptional addition of polyadenylic acid to mitochondrial RNA by a cordycepin-insensitive process. , 1974, Journal of molecular biology.

[111]  G. Attardi,et al.  The biogenesis of mitochondria. , 1970, The Biochemical journal.

[112]  E. Bartnik,et al.  Regular paper on-line at: www.actabp.pl Differential stability of mitochondrial mRNA in HeLa cells , 2005 .

[113]  C. Florentz,et al.  Mitochondrial tRNA 3' end metabolism and human disease. , 2004, Nucleic acids research.

[114]  R. Wiesner,et al.  Transient overexpression of mitochondrial transcription factor A (TFAM) is sufficient to stimulate mitochondrial DNA transcription, but not sufficient to increase mtDNA copy number in cultured cells. , 2004, Nucleic acids research.

[115]  P. Stepien,et al.  Identification of a novel human nuclear-encoded mitochondrial poly(A) polymerase. , 2004, Nucleic acids research.

[116]  P. Seibel,et al.  Glucocorticoid and thyroid hormone receptors in mitochondria of animal cells. , 2003, International review of cytology.

[117]  G. Shadel,et al.  Human mitochondrial transcription factor B1 methylates ribosomal RNA at a conserved stem-loop , 2003, Nature Genetics.

[118]  R. Wiesner,et al.  Glucocorticoid Hormone Stimulates Mitochondrial Biogenesis Specifically in Skeletal Muscle. , 2002, Endocrinology.

[119]  G. Attardi.  Animal mitochondrial DNA: an extreme example of genetic economy. , 1985, International review of cytology.

[120]  R. Perry.  Processing of RNA. , 1976, Annual review of biochemistry.