Replication and transcription of mammalian mitochondrial DNA

Experimental Physiology

Volumn 88, Issue 1, 2003, Pages 41-56

  Fernández Silva, Patricio ^a   Enriquez, José A ^a   Montoya, Julio ^a  

^a UNIVERSITY OF ZARAGOZA   (Spain)

Author keywords

[No Author keywords available]

Indexed keywords

ADENOSINE TRIPHOSPHATE; CALCIUM ION; CONTIG; DNA POLYMERASE; MITOCHONDRIAL DNA; MITOCHONDRIAL PROTEIN; POLYPEPTIDE; RIBOSOME RNA; RNA POLYMERASE; TRANSFER RNA;

ADAPTATION; AGING; ANIMAL CELL; APOPTOSIS; BIOGENESIS; CALCIUM HOMEOSTASIS; CARCINOGENESIS; CELL ORGANELLE; CONTROLLED STUDY; DNA REPLICATION; DNA TRANSCRIPTION; ENERGY YIELD; GENE EXPRESSION; GENETIC CODE; GENOME ANALYSIS; INTRON; MAMMAL; MATURATION; MITOCHONDRIAL GENETICS; MITOCHONDRION; MOLECULAR CLONING; NONHUMAN; OXIDATIVE PHOSPHORYLATION; PROTEIN ANALYSIS; REGULATORY MECHANISM; REVIEW; RNA SEQUENCE; RNA TRANSCRIPTION; SEQUENCE ANALYSIS; THERMOGENESIS;

EID: 0037252462     PISSN: 09580670     EISSN: None     Source Type: Journal    
DOI: 10.1113/eph8802514     Document Type: Review

References (166)

1. Albring M, Griffith J & Attardi G (1977). Association of a protein structure of probable membrane derivation with HeLa cell mitochondrial DNA near its origin of replication. Proc Natl Acad Sci U S A 74, 1348-1352.
2. Amalric F, Merkel C, Geldfand R & Attardi G (1978). Fractionation of mitochondrial RNA from HeLa cells by high-resolution electrophoresis under strongly denaturing conditions. J Mol Biol 118, 1-25.
3. Anderson S, Bankier AT, Barrell BG, de-Bruijn MHL, Coulson AR, Drouin J, Eperon IC, Nierlich DP, Roe BA, Sanger F, Schreier HP, Smith AJH, Stader R & Young IG (1981). Sequence and organization of the human mitochondrial genome. Nature 290, 427-465.
4. Andersson U & Scarpulla RC (2001). Pgc-1-related coactivator, a novel, serum-inducible coactivator of nuclear respiratory factor 1-dependent transcription in mammalian cells. Mol Cell Biol 21, 3738-3749.
5. Antoshechkin I & Bogenhagen DF (1995). Distinct roles for two purified factors in transcription of Xenopus mitochondrial DNA. Mol Cell Biol 15, 7032-7042.
6. Arcari P & Brownlee GG (1980). The nucleotide sequence of a small (3S) seryl-tRNA (anticodon GCU) from beef heart mitochondria. Nucleic Acids Res 8, 5207-5212.
7. Attardi G (1985). Animal mitochondrial DNA, an extreme example of genetic economy. Int Rev Cytol 93, 683-711.
8. Attardi G & Schatz G (1988). Biogenesis of mitochondria. Annu Rev Cell Biol 4, 289-333.
9. Bai YD & Attardi G (1998). 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. EMBO J 17, 4848-4858.
10. Bentley D (2002). The mRNA assembly line, transcription and processing machines in the same factory. Curr Opin Cell Biol 14, 336-342.
11. Bogenhagen DF, Applegate EF & Yoza BK (1984). Identification of a promoter for transcription of the heavy strand of human mtDNA, in vitro transcription and deletion mutagenesis. Cell 36, 1105-1113.
12. Bogenhagen DF & Clayton DA (1977). Mouse L cell mitochondrial DNA molecules are selected randomly for replication throughout the cell cycle. Cell 11, 719-727.
13. Brown TA & Clayton DA (2002). Release of replication termination controls mitochondrial DNA copy number after depletion with 2′,3′-dideoxycytidine. Nucleic Acids Res 30, 2004-2010.
14. Brown WM, George JM & Wilson AC (1979). Rapid evolution of animal mitochondrial DNA. Proc Natl Acad Sci U S A 76, 1967-1971.
15. Cantatore P, Polosa PL, Fracasso F, Flagella Z & Gadaleta MN (1986). Quantitation of mitochondrial RNA species during rat liver development, the concentration of cytochrome oxidase subunit I (Col) mRNA increase at birth. Cell Differ 19, 125-132.
16. Carmona MC, Iglesias R, Obregon MJ, Darlington GJ, Villarroya F & Giralt M (2002). Mitochondrial biogenesis and thyroid status maturation in brown fat require CCAAT/enhancer-binding protein alpha. J Biol Chem 277, 21489-21498.
17. Carrodeguas JA, Kobayashi R, Lim SE, Copeland WC & Bogenhagen DF (1999). The accessory subunit of Xenopus laevis mitochondrial DNA polymerase gamma increases processivity of the catalytic subunit of human DNA polymerase gamma and is related to class II aminoacyl-tRNA synthetases. Mol Cell Biol 19, 4039-4046.
18. Casas F, Rochard P, Rodier A, Cassarmalek I, Marchalvictorion S, Wiesner RJ, Cabello G & Wrutniak C (1999). A variant form of the nuclear triiodothyronine receptor c-ErbA alpha 1 plays a direct role in regulation of mitochondrial RNA synthesis. Mol Cell Biol 19, 7913-7924.
19. Castora FJ, Lazarus GM & Kunes D (1985). The presence of two mitochondrial DNA topoisomerases in human acute leukemia cells. Biochem Biophys Res Commun 130, 854-866.
20. Castora FJ & Simpson MV (1979). Search for a DNA gyrase in mammalian mitochondria. J Biol Chem 254, 11193-11195.
21. Chang DD & Clayton DA (1984). Precise identification of individual promoters for transcription of each strand of human mitochondrial DNA. Cell 36, 635-643.
22. Chang DD & Clayton DA (1986a). Precise asignment of the light strand promoter of mouse mitochondrial DNA: a functional promoter consist of multiple upstream domains. Mol Cell Biol 6, 3253-3261.
23. Chang DD & Clayton DA (1986b). Precise asignment of the heavy-strand promoter of mouse mitochondrial DNA: cognate start sites are not required for transcriptional initiation. Mol Cell Biol 6, 3262-3267.
24. Chomyn A & Attardi G (1992). Recent advances on mitochondrial biogenesis. In Molecular Mechanisms in Biogenesis, ed. Ernster L, chap. 20, pp. 483-509. Elsevier Science Publishers B. V., Amsterdam.
25. Christianson TW & Clayton DA (1988). A tridecamer DNA sequence supports human mitochondrial RNA 3′-end formation in vivo. Mol Cell Biol 8, 4502-4509.
26. Clayton DA (1982). Replication of animal mitochondrial DNA. Cell 28, 693-705.
27. Clayton DA (1992). Transcription and replication of animal mitochondrial DNAs. Int Rev Cytol 141, 217-232.
28. Cote J & Ruiz-Carrillo A (1993). Primers for mitochondrial DNA replication generated by endonuclease-G. Science 261, 765-769.
29. Daga A, Micol V, Hess D, Aebersold R & Attardi G (1993). Molecular characterization of the transcription termination factor from human mitochondria. J Biol Chem 268, 8123-8130.
30. Davis AF, Ropp PA, Clayton DA & Copeland WC (1996). Mitochondrial DNA polymerase gamma is expressed and translated in the absence of mitochondrial DNA maintenance and replication. Nucleic Acids Res 24, 2753-2759.
31. de Bruijn MH, Schreier PH, Eperon IC & Barrell BG (1980). A mammalian mitochondrial serine transfer RNA lacking the 'dihydrouridine' loop and stem. Nucleic Acids Res 8, 5212-5222.
32. Doda JN, Wright CT & Clayton DA (1981). Elongation of displacement-loop strands in human and mouse mitochondrial DNA is arrested near specific template sequences. Proc Natl Acad Sci U S A 78, 6116-6120.
33. Doersen CJ, Guerrier-Takada C, Altman S & Attardi G (1985). Characterization of an RNase P activity from HeLa cell mitochondria. Comparison with the cytosol RNase P activity. J Biol Chem 260, 5942-5949.
34. Dubin DT, Montoya J, Timko KD & Attardi G (1982). Sequence analysis and precise mapping of the 3′-ends of HeLa cell mitochondrial ribosomal RNAs. J Mol Biol 157, 1-19.
35. England JM, Costantino P & Attardi G (1978). Mitochondrial RNA and protein synthesis in enucleated African green monkey cells. J Mol Biol 119, 455-462.
36. Enriquez JA, Fernández-Silva P, Garrido-Perez N, Lopez-Perez MJ, Perez-Martos A & Montoya J (1999a). Direct regulation of mitochondrial RNA synthesis by thyroid hormone. Mol Cell Biol 19, 657-670.
37. Enriquez JA, Fernández-Silva P & Montoya J (1999b). Autonomous regulation in mammalian mitochondrial DNA transcription. Biol Chem 380, 737-747.
38. Enriquez JA, Fernández-Silva P, Perez-Martos A, Lopez-Perez MJ & Montoya J (1996). The synthesis of mRNA in isolated mitochondria can be maintained for several hours and is inhibited by high levels of ATP. Eur J Biochem 237, 601-610.
39. Ephrussi B, Hottinguer H & Tavlitzki J (1949). Action de l'acriflavine sur les levures. II. Etude génétique du mutant "petite colonie". Ann Inst Pasteur 76, 351-367.
40. Etten RAV, Bird JW & Clayton DA (1983). Identification of the 3′-ends of the two mouse mitochondrial ribosomal RNAs. J Biol Chem 258, 10104-10110.
41. Falkenberg M, Gaspari M, Rantanen A, Trifunovic A, Larsson NG & Gustafsson CM (2002). Mitochondrial transcription factors B1 and B2 activate transcription of human mtDNA. Nat Genet 31, 289-294.
42. Fan L, Sanschagrin PC, Kaguni LS & Kuhn LA (1999). The accessory subunit of mtDNA polymerase shares structural homology with aminoacyl-tRNA synthetases. Implications for a dual role as a primer recognition factor and processivity clamp. Proc Nat Acad Sci U S A 96, 9527-9532.
43. Farr CL, Wang Y & Kaguni LS (1999). Functional interactions of mitochondrial DNA polymerase and single-stranded DNA-binding protein. Template-primer DNA binding and initiation and elongation of DNA strand synthesis. J Biol Chem 274, 14779-14785.
44. Fernández-Silva P, Martinez-Azorin F, Micol V & Attardi G (1997). 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. EMBO J 16, 1066-1079.
45. Fernández-Silva P, Petruzzella V, Fracasso F, Gadaleta MN & Cantatore P (1991). Reduced synthesis of mt RNA in isolated mitochondria of senescent rat brain. Biochem Biophys Res Commun 176, 645-653.
46. Fisher RP & Clayton DA (1985). A transcription factor required for promoter recognition by human mitochondrial RNA polymerase. J Biol Chem 260, 11330-11338.
47. Fisher RP & Clayton, DA (1988). Purification and characterization of human mitochondrial transcription factor 1. Mol Cell Biol 8, 3496-3509.
48. Fisher RP, Lisowsky T, Parisi MA & Clayton DA (1992). DNA wrapping and bending by a mitochondrial high mobility group-like transcriptional activator protein. J Biol Chem 267, 3358-3367.
49. Fisher RP, Topper JN & Clayton DA (1987). Promoter selection in human mitochondria involves binding of a transcription factor to orientation-independent upstream regulatory elements. Cell 50, 247-258.
50. Gadaleta MN, Petruzzella V, Fracasso F, Fernández-Silva P & Cantatore P (1990a). Acetyl-L-carnitine increases cytochrome oxidase subunit I mRNA content in hypothyroid rat liver. FEBS Lett 277, 191-193.
51. Gadaleta MN, Petruzella V, Renis M, Fracasso F & Cantatore P (1990b). Reduced transcription of mitochondrial DNA in the senescent rat. Tissue dependence and effect of L-carnitine. Eur J Biochem 187, 501-506.
52. Gaines G & Attardi G (1984). Highly efficient RNA-synthesizing system that uses isolated human mitochondria, new initiation events and in vivo-like processing patterns. Mol Cell Biol 4, 1605-1617.
53. Gaines G, Rosi C & Attardi G (1987). Markedly different ATP requirements for rRNA synthesis and mtDNA light strand transcription versus mRNA synthesis in isolated human mitochondria. J Biol Chem 262, 1907-1915.
54. Garstka HL, Facke M, Escribano JR & Wiesner RJ (1994). Stoichiometry of mitochondrial transcripts and regulation of gene expression by mitochondrial transcription factor A. Biochem Biophys Res Commun 200, 619-626.
55. Gensler S, Weber K, Schmitt WE, Perez-Martos A, Enriquez JA, Montoya J & Wiesner RJ (2001). Mechanism of mammalian mitochondrial DNA replication, import of mitochondrial transcription factor A into isolated mitochondria stimulates 7S DNA synthesis. Nucleic Acids Res 29, 3657-3663.
56. Ghivizzani SC, Madsen CS, Nelen MR, Ammini CV & Hauswirth WW (1994). In organello footprint analysis of human mitochondrial DNA, human mitochondrial transcription factor A interactions at the origin of replication. Mol Cell Biol 14, 7717-7730.
57. Goglia F, Moreno M & Lanni A (1999). Action of thyroid hormones at the cellular level, the mitochondrial target. FEBS Lett 452, 115-120.
58. Gordon JW, Rungi AA, Inagaki H & Hood DA (2001). Effects of contractile activity on mitochondrial transcription factor A expression in skeletal muscle. J Appl Physiol 90, 389-396.
59. Graves SW, Johnson AA & Johnson KA (1998). Expression, purification, and initial kinetic characterization of the large subunit of the human mitochondrial DNA polymerase. Biochemistry 37, 6050-6058.
60. Gray H & Wong TW (1992). Purification and identification of subunit structure of the human mitochondrial DNA polymerase. J Biol Chem 267, 5835-5841.
61. Hehman GL & Hauswirth WW (1992). DNA helicase from mammalian mitochondria. Proc Natl Acad Sci U S A 89, 8562-8566.
62. Helm M, Brule H, Friede D, Giege R, Putz D & Florentz C (2000). Search for characteristic structural features of mammalian mitochondrial tRNAs. RNA 6, 1356-1379.
63. Herzig RP, Scacco S & Scarpulla RC (2000). Sequential serum-dependent activation of CREB and NRF-1 leads to enhanced mitochondrial respiration through the induction of cytochrome c. J Biol Chem 275, 13134-13141.
64. Hess JF, Parisi MA, Bennett JL & Clayton DA (1991). Impairment of mitochondrial transcription termination by a point mutation associated with the MELAS subgroup of mitochondrial encephalomyopathies. Nature 351, 236-239.
65. Hevner RF & Wong-Riley MT (1993). Mitochondrial and nuclear gene expression for cytochrome oxidase subunits are disproportionately regulated by functional activity in neurons. J Neurosci 13, 1805-1819.
66. Hixson JE, Wong TW & Clayton DA (1986). Both the conserved stem-loop and divergent 5′-flanking sequences are required for initiation at the human mitochondrial origin of light-strand DNA replication. J Biol Chem 261, 2384-2390.
67. Hobbs AE, Srinivasan M, McCaffery JM & Jensen RE (2001). Mmm1p, a mitochondrial outer membrane protein, is connected to mitochondrial DNA (mtDNA) nucleoids and required for mtDNA stability. J Cell Biol 152, 401-410.
68. Hofhaus G & Attardi G (1995). Efficient selection and characterization of mutants of a human cell line which are defective in mitochondrial DNA-encoded subunits of respiratory NADH dehydrogenase. Mol Cell Biol 15, 964-974.
69. Hoke GD, Pavco PA, Ledwith BJ & Tuyle GCV (1990). Structural and functional studies of the rat mitochondrial single-stranded DNA binding protein P16. Arch Biochem Biophys 282, 116-124.
70. Holt IJ, Harding AE, Petty RKH & Morgan-Hughes JA (1990). A new mitochondrial disease associated with mitochondrial DNA heteroplasmy. Am J Hum Genet 46, 428-433.
71. Holt IJ, Lorimer HE & Jacobs HT (2000). Coupled leading- and lagging-strand synthesis of mammalian mitochondrial DNA. Cell 100, 515-524.
72. Hood DA, Zak R & Pette D (1989). Chronic stimulation of rat skeletal muscle induces coordinate increases in mitochondrial and nuclear mRNAs of cytochrome-c-oxidase subunits. Eur J Biochem 179, 275-280.
73. Izquierdo JM, Ricart J, Ostronoff LK, Egea G & Cuezva JM (1995). Changing patterns of transcriptional and post-transcriptional control of beta-F-1-ATPase gene expression during mitochondrial biogenesis in liver. J Biol Chem 270, 10342-10350.
74. Jacobs HT, Lehtinen SK & Spelbrink JN (2000). No sex please, we're mitochondria, a hypothesis on the somatic unit of inheritance of mammalian mtDNA. Bioessays 22, 564-572.
75. Jenuth JP, Peterson AC & Shoubridge EA (1997). Tissue-specific selection for different mtDNA genotypes in heteroplasmic mice. Nat Genet 16, 93-95.
76. Kadenbach B, Possekel S, Huttemann M & Arnold S (1998). Biochemical defects and genetic abnormalities in cytochrome c oxidase of patients with Leigh syndrome. Biofactors 7, 273-276.
77. Kaufman BA, Newman SM, Hallberg RL, Slaughter CA, Perlman PS & Butow RA (2000). In organello formaldehyde crosslinking of proteins to mtDNA, identification of bifunctional proteins. Proc Natl Acad Sci U S A 97, 7772-7777.
78. Kaukonen J, Juselius JK, Tiranti V, Kyttala A, Zeviani M, Comi GP, Keranen S, Peltonen L & Suomalainen A (2000). Role of adenine nucleotide translocator 1 in mtDNA maintenance. Science 289, 782-785.
79. Kosovsky M & Soslau G (1993). Immunological identification of human platelet mitochondrial DNA topoisomerase-I. Biochim Biophys Acta 1164, 101-107.
80. Kroemer G & Reed JC (2000). Mitochondrial control of cell death. Nat Med 6, 513-519.
81. Kruse B, Narasimhan N & Attardi G (1989). Termination of transcription in human mitochondria, identification and purification of a DNA binding protein factor that promotes termination. Cell 58, 391-397.
82. Lang BF, Gray MW & Burger G (1999). Mitochondrial genome evolution and the origin of eukaryotes. Annu Rev Genet 33, 351-397.
83. Larsson NG, Wang JM, Wilhelmsson H, Oldfors A, Rustin P, Lewandoski M, Barsh GS & Clayton DA (1998). Mitochondrial transcription factor A is necessary for mtDNA maintenance and embryogenesis in mice. Nat Genet 18, 231-236.
84. Lecrenier N & Foury F (2000). New features of mitochondrial DNA replication system in yeast and man. Gene 246, 37-48.
85. Lee DY & Clayton D (1996). Properties of a primer RNA-DNA hybrid at the mouse mitochondrial DNA leading-strand origin of replication. J Biol Chem 271, 24262-24269.
86. Lefai E, Fernández-Moreno MA, Alahari A, Kaguni LS & Garesse R (2000). Differential regulation of the catalytic and accessory subunit genes of Drosophila mitochondrial DNA polymerase. J Biol Chem 275, 33123-33133.
87. Lightowlers RN, Chinnery PF, Turnbull DM & Howell N (1997). Mammalian mitochondrial genetics, heredity, heteroplasmy and disease. Trends Genet 13, 450-455.
88. Lim SE, Longley MJ & Copeland WC (1999). The mitochondrial p55 accessory subunit of human DNA polymerase gamma enhances DNA binding, promotes processive DNA synthesis, and confers N-ethylmaleimide resistance. J Biol Chem 274, 38197-38203.
89. Loguercio-Polosa P & Attardi G (1991). Distinctive pattern and transcriptional control of mitochondrial protein synthesis in rat brain synaptic endings. J Biol Chem 266, 10011-10017.
90. Longley MJ, Nguyen D, Kunkel TA & Copeland WC (2001). The fidelity of human DNA polymerase gamma with and without exonucleolytic proofreading and the p55 accessory subunit. J Biol Chem 276, 38555-38562.
91. McCulloch V, Seidel-Rogol BL & Shadel GS (2002). A human mitochondrial transcription factor is related to RNA adenine methyltransferases and binds s-adenosylmethionine. Mol Cell Biol 22, 1116-1125.
92. Madsen CS Ghivizzani SC & Hauswirth WW (1993). Protein binding to a single termination-associated sequence in the mitochondrial DNA D-loop region. Mol Cell Biol 13, 2162-2171.
93. Marchington DR, Macaulay V, Hartshorne GM, Barlow D & Poulton J (1998). Evidence from human oocytes for a genetic bottleneck in an mtDNA disease. Am J Hum Genet 63, 769-775.
94. Martin I, Vinas O, Mampel T, Iglesias R & Villarroya F (1993). 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. Biochem J 296, 231-234.
95. Michikawa Y, Mazzucchelli F, Bresolin N, Scarlato G & Attardi G (1999). Aging-dependent large accumulation of point mutations in the human mtDNA control region for replication. Science 286, 774-779.
96. Micol V, Fernández-Silva P & Attardi G (1996). Isolation and assay of mitochondrial transcription termination factor from human cells. Methods Enzymol 264, 158-173.
97. Micol V, Fernández-Silva P & Attardi G (1997). Functional analysis of in vivo and in organello footprinting of HeLa cell mitochondrial DNA in relationship to ATP and ethidium bromide effects on transcription. J Biol Chem 272, 18896-18904.
98. Miyakawa I, Sando N, Kawano S, Nakamura S & Kuroiwa T (1987). Isolation of morphologically intact mitochondrial nucleoids from the yeast, Saccharomyces cerevisiae. J Cell Sci 88, 431-439.
99. Montoya J, Christianson T, Levens D, Rabinowitz M & Attardi G (1982). Identification of initiation sites for heavy strand and light strand transcription in human mitochondrial DNA. Proc Natl Acad Sci U S A 79, 7195-7199.
100. Montoya J, Gaines GL & Attardi G (1983). The pattern of transcription of the human mitochondrial rRNA genes reveals two overlapping transcription units. Cell 34, 151-159.
101. Montoya J, Ojala D & Attardi G (1981). Distinctive features of the 5′-terminal sequences of the human mitochondrial mRNAs. Nature 290, 465-470.
102. Montoya J, Perez-Martos A, Garstka HL & Wiesner RJ (1997). Regulation of mitochondrial transcription by mitochondrial transcription factor A. Mol Cell Biochem 174, 227-230.
103. Moraes CT (2001). What regulates mitochondrial DNA copy number in animal cells? Trends Genet 17, 199-205.
104. Moraes CT, Kenyon L & Hao HL (1999). Mechanisms of human mitochondrial DNA maintenance, the determining role of primary sequence and length over function. Mol Biol Cell 10, 3345-3356.
105. Mutvei A, Kuzela S & Nelson BD (1989). Control of mitochondrial transcription by thyroid hormone. Eur J Biochem 180, 235-240.
106. Nagaike T, Suzuki T, Tomari Y, Takemotohori C, Negayama F, Watanabe K & Ueda T (2001). Identification and characterization of mammalian mitochondrial tRNA nucleotidyltransferases. J Biol Chem 276, 40041-40049.
107. Nass MMK & Nass S (1963). Intramitochondrial fibers with DNA characteristics. J Cell Biol 19, 593-629.
108. Nie F & Wong-Riley MT (1996). Mitochondrial- and nuclear-encoded subunits of cytochrome oxidase in neurons, differences in compartmental distribution, correlation with enzyme activity, and regulation by neuronal activity. J Comp Neurol 373, 139-155.
109. Nishino I, Spinazzola A & Hirano M (1999). Thymidine phosphorylase gene mutations in MNGIE, a human mitochondrial disorder. Science 283, 689-692.
110. Ojala D, Montoya J & Attardi G (1981). tRNA punctuation model of RNA processing in human mitochondria. Nature 290, 470-474.
111. Ostronoff LK, Izquierdo JM, Enriquez JA, Montoya J & Cuezva JM (1996). Transient activation of mitochondrial translation regulates the expression of the mitochondrial genome during mammalian mitochondrial differentiation. Biochem J 316, 183-191.
112. Overly CC, Rieff HI & Hollenbeck PJ (1996). Organelle motility and metabolism in axons vs dendrites of cultured hippocampal neurons. J Cell Sci 109, 971-980.
113. Papa S, Sardanelli AM, Scacco S & Technikova-Dobrova Z (1999). cAMP-dependent protein kinase and phosphoproteins in mammalian mitochondria. An extension of the cAMP-mediated intracellular signal transduction. FEBS Lett 444, 245-249.
114. Parisi MA & Clayton DA (1991). Similarity of human mitochondrial transcription factor 1 to high mobility group proteins. Science 252, 965-969.
115. Penta JS, Johnson FM, Wachsman JT & Copeland C (2001). Mitochondrial DNA in human malignancy. Mutat Res 488, 119-133.
116. Perez-Jannotti RM, Klein SM & Bogenhagen DF (2001). Two forms of mitochondrial DNA ligase III are produced in Xenopus laevis oocytes. J Biol Chem 276, 48978-48987.
117. Pinz KG & Bogenhagen DF (1998). Efficient repair of abasic sites in DNA by mitochondrial enzymes. Mol Cell Biol 18, 1257-1265.
118. Preiss T, Sang AE, Chrzanowska-Lightowlers ZM & Lightowlers RN (1995). The mRNA-binding protein COLBP is glutamate dehydrogenase. FEBS Lett 367, 291-296.
119. Prieto-Martín A, Montoya J & Martínez-Azorín F (2001). A study on the human mitochondrial RNA polymerase activity points to existence of a transcription factor B-like protein. FEBS Lett 503, 51-55.
120. Puranam RS & Attardi G (2001). The RNase P associated with HeLa cell mitochondria contains an essential RNA component identical in sequence to that of the nuclear RNase P. Mol Cell Biol 21, 548-561.
121. Richter C, Park JW & Ames BN (1988). Normal oxidative damage to mitochondrial and nuclear DNA is extensive. Proc Natl Acad Sci U S A 85, 6465-6467.
122. Roberti M, Musicco C, Polosa PL, Gadaleta MN & Cantatore P (1996). DNA-helicase activity from sea urchin mitochondria. Biochem Biophys Res Commun 219, 134-139.
123. Rossmanith W, Tullo A, Potuschak T, Karwan R & Sbisa E (1995). Human mitochondrial tRNA processing. J Biol Chem 270, 12885-12891.
124. Scarpulla RC (1997). Nuclear control of respiratory chain expression in mammalian cells. J Bioenerg Biomembr 29, 109-119.
125. Scarpulla RC (2002). Nuclear activators and coactivators in mammalian mitochondrial biogenesis. Biochim Biophys Acta 1576, 1-14.
126. Schultz RA, Swoap SJ, McDaniel LD, Zhang B, Koon EC, Garry DJ, Li K & Williams RS (1998). Differential expression of mitochondrial DNA replication factors in mammalian tissues. J Biol Chem 273, 3447-3451.
127. Schwartz M & Vissing J (2002). Paternal inheritance of mitochondrial DNA. N Engl J Med 347, 576-580.
128. Shadel GS & Clayton DA (1997). Mitochondrial DNA maintenance in vertebrates. Annu Rev Biochem 66, 409-435.
129. Shang J & Clayton DA (1994). Human mitochondrial transcription termination exhibits RNA polymerase independence and biased bipolarity in vitro. J Biol Chem 269, 29112-29120.
130. Shay JW, Pierce DJ & Werbin H (1990). Mitochondrial DNA copy number is proportional to total cell DNA under variety of growth conditions. J Biol Chem 264, 14802-14807.
131. Shen EL & Bogenhagen DF (2001). Developmentally-regulated packaging of mitochondrial DNA by the HMG-box protein mtTFA during Xenopus oogenesis. Nucleic Acids Res 29, 2822-2828.
132. Shoubridge EA (2002). The ABCs of mitochondrial transcription. Nat Genet 31, 227-228.
133. Shuster RC, Rubenstein AJ & Wallace DC (1988). Mitochondrial DNA in anucleate human blood cells. Biochem Biophys Res Commun 155, 1360-1365.
134. Spelbrink JN, Li FY, Tiranti V, Nikali K, Yuan QP, Tariq M, Wanrooij S, Garrido N, Comi G, Morandi L, Santoro L, Toscano A, Fabrizi GM, Somer H, Croxen R, Beeson D, Poulton J, Suomalainen A, Jacobs HT, Zeviani M & Larsson C (2001). Human mitochondrial DNA deletions associated with mutations in the gene encoding Twinkle, a phage T7 gene 4-like protein localized in mitochondria. Nat Genet 28, 223-231.
135. Stoneking M (1994). Mitochondrial DNA and human evolution. J Bioenerg Biomembr 26, 251-259.
136. Sutovsky P, Moreno RD, Ramalho-Santos J, Dominko T, Simerly C & Schatten G (2000). Ubiquitinated sperm mitochondria, selective proteolysis, and the regulation of mitochondrial inheritance in mammalian embryos. Biol Reprod 63, 582-590.
137. Sutovsky P, Moreno RD & Schatten G (1999). Ubiquitin tag for sperm mitochondria. Nature 402, 371.
138. Taanman JW (1999). The mitochondrial genome, structure, transcription, translation and replication. Biochim Biophys Acta 1410, 103-123.
139. Takamatsu C, Umeda S, Ohsato T, Ohno T, Abe Y, Fukuoh A, Shinagawa H, Hamasaki N & Kang DC (2002). Regulation of mitochondrial D-loops by transcription factor A and single-stranded DNA-binding protein. EMBO Rep 3, 451-456.
140. Tang YY, Schon EA, Wilichowski E, Vazquezmemije ME, Davidson E & King MP (2000). Rearrangements of human mitochondrial DNA (mtDNA), new insights into the regulation of mtDNA copy number and gene expression. Mol Biol Cell 11, 1471-1485.
141. Tiranti V, Savoia A, Forti F, Dapolito MF, Centra M, Racchi M & Zeviani M (1997). Identification of the gene encoding the human mitochondrial RNA polymerase (h-mtRPOL) by cyberscreening of the expressed sequence tags database. Hum Mol Genet 6, 615-625.
142. Tsiriyotis C, Spandidos DA & Sekeris CE (1997). The mitochondrion as a primary site of action of glucocorticoids, mitochondrial nucleotide sequences, showing similarity to hormone response elements, confer dexamethasone inducibility to chimaeric genes transfected in LATK(-) cells. Biochem Biophys Res Commun 235, 349-354.
143. Van Dyck E, Foury F, Stillman B & Brill SJ (1992). A single-stranded DNA binding protein required for mitochondrial DNA replication in S. cerevisiae is homologous to E. coli SSB. EMBO J 11, 3421-3430.
144. VanItallie CM (1990). Thyroid hormone and dexamethasone increase the levels of a messenger ribonucleic acid for a mitochondrially encoded subunit but not for a nuclear-encoded subunit cytocrome c oxidase. Endocrinology 127, 55-62.
145. Virbasius JV & Scarpulla RC (1994). Activation of the human transcription factor A gene by nuclear respiratory factors - a potential regulatory link between nuclear and mitochondrial gene expression in organelle biogenesis. Proc Natl Acad Sci U S A 91, 1309.
146. Walberg MW & Clayton DA (1981). Sequence and properties of the human KB cell and mouse L cell D-loop regions of mitochondrial DNA. Nucleic Acids Res 9, 5411-5421.
147. Walberg MW & Clayton DA (1983). In vitro transcription of human mitochondrial DNA, identification of specific light strand transcripts from the displacement loop region. J Biol Chem 258, 1268-1275.
148. Wallace DC (1992). Diseases of the mitochondrial DNA. Annu Rev Biochem 61, 1175-1212.
149. Wallace DC (1997). Mitochondrial DNA in aging and disease. Sci Am 277, 40-47.
150. Wallace DC, Brown MD & Lott MT (1999). Mitochondrial DNA variation in human evolution and disease. Gene 238, 211-230.
151. Weber K, Bruck P, Mikes Z, Kupper JH, Klingenspor M & Wiesner RJ (2002). Glucocorticoid hormone stimulates mitochondrial biogenesis specifically in skeletal muscle. Endocrinology 143, 177-184.
152. Wiesner RJ, Aschenbrenner V, Ruegg JC & Zak R (1994). Coordination of nuclear and mitochondrial gene expression during the development of cardiac hypertrophy in rats. Am J Physiol 267, C229-235.
153. Wiesner RJ, Kurowski TT & Zak R (1992a). Regulation by thyroid hormones of nuclear and mitochondrial genes encoding subunits of cytochrome-c oxidase in rat liver and skeletal muscle. Mol Endocrinol 6, 1458-1467.
154. Wiesner RJ, Ruegg JC & Morano I (1992b). Counting target molecules by exponential polymerase chain reaction, copy number of mitochondrial DNA in rat tissues. Biochim Biophys Acta 183, 553-559.
155. Williams RS (1986). Mitochondrial gene expression in mammalian striated muscle. Evidence that variation in gene dosage is the major regulatory event. J Biol Chem 261, 12390-12394.
156. Wolstenholme DR (1992). Animal mitochondrial DNA, structure and evolution. Int Rev Cytol 141, 173-216.
157. Wong TW & Clayton DA (1985). In vitro replication of human mitochondrial DNA, accurate initiation at the origin of light-strand synthesis. Cell 42, 952-958.
158. Wong TW & Clayton DA (1986). DNA primase of human mitochondria is associated with structural RNA that is essential for enzymatic activity. Cell 45, 817-825.
159. Wrutniak C, Cassar-Malek I, Marchal S, Rascle A, Heusser S, Keller JM, Flechon J, Dauca M, Samarut J, Ghysdael J & Cabello G (1995). A 43-kDa protein related to c-Erb A α 1 is located in the mitochondrial matrix of rat liver. J Biol Chem 270, 16347-16354.
160. Wu Z, Puigserver P, Andersson U, Zhang C, Adelmant G, Mootha V, Troy A, Cinti S, Lowell B, Scarpulla RC & Spiegelman BM (1999). Mechanisms controlling mitochondrial biogenesis and respiration through the thermogenic coactivator PGC-1. Cell 98, 115-124.
161. Xu BJ & Clayton DA (1992). Assignment of a yeast protein necessary for mitochondrial transcription initiation. Nucleic Acids Res 20, 1053-1059.
162. Xu BJ & Clayton DA (1996). RNA-DNA hybrid formation at the human mitochondrial heavy-strand origin ceases at replication start sites. An implication for RNA-DNA hybrids serving as primers. EMBO J 15, 3135-3143.
163. Yoshida Y, Izumi H, Ise T, Uramoto H, Torigoe T, Ishiguchi H, Murakami T, Tanabe M, Nakayama Y, Itoh H, Kasai H & Kohno K (2002). Human mitochondrial transcription factor A binds preferentially to oxidatively damaged DNA. Biochem Biophys Res Commun 295, 945-951.
164. Yoza BK & Bogenhagen DF (1984). Identification and in vitro capping of a primary transcript of human mitochondrial DNA. J Biol Chem 259, 3909-3915.
165. Zeviani M & Antozzi C (1997). Mitochondrial disorders. Mol Hum Reprod 3, 133-148.
166. Zhang HL, Barcelo JM, Lee B, Kohlhagen G, Zimonjic DB, Popescu NC & Pommier Y (2001). Human mitochondrial topoisomerase I. Proc Nat Acad Sci U S A 98, 10608-10613.