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In vivo functional analysis of the human mitochondrial DNA polymerase POLG expressed in cultured human cells

Bibliographic reference Spelbrink, Johannes ; Toivonen, Janne ; Hakkaart, Gerrit ; Kurkela, Johanna ; Cooper, Helen ; et. al. In vivo functional analysis of the human mitochondrial DNA polymerase POLG expressed in cultured human cells. In: Journal of Biological Chemistry, Vol. 275, no. 32, p. 24818-24828 (2000)
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  1. Anderson S., Bankier A. T., Barrell B. G., de Bruijn M. H. L., Coulson A. R., Drouin J., Eperon I. C., Nierlich D. P., Roe B. A., Sanger F., Schreier P. H., Smith A. J. H., Staden R., Young I. G., Sequence and organization of the human mitochondrial genome, 10.1038/290457a0
  2. Rovio Anja, Tiranti Valeria, Bednarz Amy L, Suomalainen Anu, Spelbrink Johannes N, Lecrenier Nicolas, Melberg Atle, Zeviani Massimo, Poulton Joanna, Foury Françoise, Jacobs Howard T, Analysis of the trinucleotide CAG repeat from the human mitochondrial DNA polymerase gene in healthy and diseased individuals, 10.1038/sj.ejhg.5200244
  3. Bernad Antonio, Blanco Luis, Lázaro JoséM., Martín Gil, Salas Margarita, A conserved 3′→5′ exonuclease active site in prokaryotic and eukaryotic DNA polymerases, 10.1016/0092-8674(89)90883-0
  4. Foury, EMBO J., 11, 2717 (1992)
  5. Hu Jingping, Vanderstraeten Sylvie, Foury Françoise, Isolation and characterization of ten mutator alleles of the mitochondrial DNA polymerase-encoding MIP1 gene from Saccharomyces cerevisiae, 10.1016/0378-1119(95)00215-r
  6. Longley Matthew J., Ropp Philip A., Lim Susan E., Copeland William C., Characterization of the Native and Recombinant Catalytic Subunit of Human DNA Polymerase γ:  Identification of Residues Critical for Exonuclease Activity and Dideoxynucleotide Sensitivity, 10.1021/bi980772w
  7. Vanderstraeten Sylvie, Van den Brûle Sybille, Hu Jingping, Foury Françoise, The Role of 3′-5′ Exonucleolytic Proofreading and Mismatch Repair in Yeast Mitochondrial DNA Error Avoidance, 10.1074/jbc.273.37.23690
  8. Farr Carol L., Wang Yuxun, Kaguni Laurie S., Functional Interactions of Mitochondrial DNA Polymerase and Single-stranded DNA-binding Protein : TEMPLATE-PRIMER DNA BINDING AND INITIATION AND ELONGATION OF DNA STRAND SYNTHESIS, 10.1074/jbc.274.21.14779
  9. Mikhailov Victor S., Bogenhagen Daniel F., Effects ofXenopus laevisMitochondrial Single-stranded DNA-binding Protein on Primer-Template Binding and 3′→5′ Exonuclease Activity of DNA Polymerase γ, 10.1074/jbc.271.31.18939
  10. Mikhailov Victor S., Bogenhagen Daniel F., Termination within Oligo(dT) Tracts in Template DNA by DNA Polymerase γ Occurs with Formation of a DNA Triplex Structure and Is Relieved by Mitochondrial Single-stranded DNA-binding Protein, 10.1074/jbc.271.48.30774
  11. Williams Andrea J., Kaguni Laurie S., Stimulation ofDrosophilaMitochondrial DNA Polymerase by Single-stranded DNA-binding Protein, 10.1074/jbc.270.2.860
  12. Wang Yuxun, Farr Carol L., Kaguni Laurie S., Accessory Subunit of Mitochondrial DNA Polymerase fromDrosophilaEmbryos : CLONING, MOLECULAR ANALYSIS, AND ASSOCIATION IN THE NATIVE ENZYME, 10.1074/jbc.272.21.13640
  13. Clayton David A., Replication of animal mitochondrial DNA, 10.1016/0092-8674(82)90049-6
  14. Carrodeguas José A., Kobayashi Ryuji, Lim Susan E., Copeland William C., Bogenhagen Daniel F., The Accessory Subunit ofXenopus laevisMitochondrial DNA Polymerase γ Increases Processivity of the Catalytic Subunit of Human DNA Polymerase γ and Is Related to Class II Aminoacyl-tRNA Synthetases, 10.1128/mcb.19.6.4039
  15. Fan L., Sanschagrin P. C., Kaguni L. S., Kuhn L. A., 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, 10.1073/pnas.96.17.9527
  16. Graves Steven W., Johnson Allison A., Johnson Kenneth A., Expression, Purification, and Initial Kinetic Characterization of the Large Subunit of the Human Mitochondrial DNA Polymerase†, 10.1021/bi972685u
  17. Gray, J. Biol. Chem., 267, 5835 (1992)
  18. Longley M. J., Prasad R., Srivastava D. K., Wilson S. H., Copeland W. C., Identification of 5'-deoxyribose phosphate lyase activity in human DNA polymerase   and its role in mitochondrial base excision repair in vitro, 10.1073/pnas.95.21.12244
  19. Dunbar D. R., Moonie P. A., Zeviani M., Holt I. J., Complex I deficiency is Associated with 3243G:C Mitochondrial DNA in Osteosarcoma Cell Cybrids, 10.1093/hmg/5.1.123
  20. Woriax Velinda L., Burkhart Will, Spremulli Linda L., Cloning, sequence analysis and expression of mammalian mitochondrial protein synthesis elongation factor Tu, 10.1016/0167-4781(95)00176-x
  21. LAEMMLI U. K., Cleavage of Structural Proteins during the Assembly of the Head of Bacteriophage T4, 10.1038/227680a0
  22. Schägger Hermann, von Jagow Gebhard, Tricine-sodium dodecyl sulfate-polyacrylamide gel electrophoresis for the separation of proteins in the range from 1 to 100 kDa, 10.1016/0003-2697(87)90587-2
  23. Zeviani Massimo, Amati Patrizia, Comi Giacomo, Fratta Giovanni, Mariotti Caterina, Tiranti Valeria, Searching for genes affecting the structural integrity of the mitochondrial genome, 10.1016/0925-4439(95)00022-v
  24. Towbin H., Staehelin T., Gordon J., Electrophoretic transfer of proteins from polyacrylamide gels to nitrocellulose sheets: procedure and some applications., 10.1073/pnas.76.9.4350
  25. Nijtmans Leo G.J., Spelbrink Johannes N., Van Galen Mieke J.M., Zwaan Mark, Klement Petr, Van den Bogert Coby, Expression and fate of the nuclearly encoded subunits of cytochrome-c oxidase in cultured human cells depleted of mitochondrial gene products, 10.1016/0167-4889(94)00203-q
  26. Spelbrink Johannes N., Van Oost Bernard A., Bogert Coby Van den, The relationship between mitochondrial genotype and mitochondrial phenotype in lymphoblasts with a heteroplasmic mtDNA deletion, 10.1093/hmg/3.11.1989
  27. Morrissey James H., Silver stain for proteins in polyacrylamide gels: A modified procedure with enhanced uniform sensitivity, 10.1016/0003-2697(81)90783-1
  28. Fernandez-Silva P., 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, 10.1093/emboj/16.5.1066
  29. Hauswirth W. W. Lim L. O. Turner G. (1987) in Mitochondria, a Practical Approach, eds Darley-Usmar V. M. Rickwood D. Wilson M. T. (IRL Press, Oxford), pp 176–177.
  30. Van den Bogert C., Pennings A., Dekker H.L., Luciaková K., Boezeman J.B.M., Sinjorgo K.M.C., Quantification of mitochondrial proteins in cultured cells by immuno-flow cytometry, 10.1016/0925-4439(91)90090-v
  31. Shevchenko Andrej, Wilm Matthias, Vorm Ole, Mann Matthias, Mass Spectrometric Sequencing of Proteins from Silver-Stained Polyacrylamide Gels, 10.1021/ac950914h
  32. Lehtinen, Genetics, 154, 363 (2000)
  33. Spelbrink J. N., Zwart Rob, Van Galen Mieke J. M., Van den Bogert C., Preferential amplification and phenotypic selection in a population of deleted and wild-type mitochondrial DNA in cultured cells, 10.1007/s002940050255
  34. Lecrenier Nicolas, Van Der Bruggen Pierre, Foury Françoise, Mitochondrial DNA polymerases from yeast to man: a new family of polymerases, 10.1016/s0378-1119(96)00663-4
  35. Meziane Abdellatif El, Lehtinen Sanna K., Hance Nicole, Nijtmans Leo G. J., Dunbar Donald, Holt Ian J., Jacobs Howard T., A tRNA suppressor mutation in human mitochondria, 10.1038/ng0498-350
  36. Polesky, J. Biol. Chem., 267, 8417 (1992)
  37. Schägger Hermann, von Jagow Gebhard, Blue native electrophoresis for isolation of membrane protein complexes in enzymatically active form, 10.1016/0003-2697(91)90094-a
  38. Arnold I., Yeast mitochondrial F1F0-ATP synthase exists as a dimer: identification of three dimer-specific subunits, 10.1093/emboj/17.24.7170
  39. Nicke A., P2X1 and P2X3 receptors form stable trimers: a novel structural motif of ligand-gated ion channels, 10.1093/emboj/17.11.3016
  40. King M., Attardi G, Human cells lacking mtDNA: repopulation with exogenous mitochondria by complementation, 10.1126/science.2814477
  41. Schultz Roger A., Swoap Steven J., McDaniel Lisa D., Zhang Bingqing, Koon E. Colin, Garry Daniel J., Li Kang, Williams R. Sanders, Differential Expression of Mitochondrial DNA Replication Factors in Mammalian Tissues, 10.1074/jbc.273.6.3447
  42. Williams, J. Biol. Chem., 261, 2390 (1986)
  43. Larsson N.-G., Oldfors A., David Garman J., Barsh G. S., Clayton D. A., Down-Regulation of Mitochondrial Transcription Factor a During Spermatogenesis in Humans, 10.1093/hmg/6.2.185
  44. Larsson Nils-Göran, Wang Jianming, Wilhelmsson Hans, Oldfors Anders, Rustin Pierre, Lewandoski Mark, Barsh Gregory S., Clayton David A., Mitochondrial transcription factor A is necessary for mtDNA maintance and embryogenesis in mice, 10.1038/ng0398-231
  45. André Paulo, Kim Andrea, Khrapko Konstantin, Thilly William G., Fidelity and Mutational Spectrum ofPfuDNA Polymerase on a Human Mitochondrial DNA Sequence, 10.1101/gr.7.8.843
  46. Ropp Philip A., Copeland William C., Cloning and Characterization of the Human Mitochondrial DNA Polymerase, DNA Polymerase γ, 10.1006/geno.1996.0490
  47. Shoubridge E.A., degSegregation of Mitochondrial DNAs Carrying a Pathogenic Point Mutation (tRNAleu3243) in Cybrid Cells, 10.1006/bbrc.1995.2115
  48. Marcelino, Cancer Res., 58, 2857 (1998)
  49. Polesky, J. Biol. Chem., 265, 14579 (1990)
  50. Fuenmayor Jahely, Zhang Jun, Ruyechan William, Williams Noreen, Identification and Characterization of Two DNA Polymerase Activities Present in Trypanosoma brucei Mitochondria, 10.1111/j.1550-7408.1998.tb05091.x
  51. Nielsen-Preiss Sheila M., Low Robert L., Identification of a β-like DNA Polymerase Activity in Bovine Heart Mitochondria, 10.1006/abbi.1999.1590
  52. Turnbull Douglass M., Andrews Richard M., Kubacka Iwona, Chinnery Patrick F., Lightowlers Robert N., Howell Neil, 10.1038/13779
  53. Hess Jürg, Burkhard Pierre, Morris Michael, Lalioti Maria, Myers Peter, Hadengue Antoine, Ischaemic colitis due to mitochondrial cytopathy, 10.1016/s0140-6736(95)91251-7
  54. Walker Robert L., Anziano Paul, Meltzer Paul S., A PAC Containing the Human Mitochondrial DNA Polymerase Gamma Gene (POLG) Maps to Chromosome 15q25, 10.1006/geno.1996.4580
  55. Foury, J. Biol. Chem., 264, 20552 (1989)
  56. Blanco L., Bernad A., Salas M., MIP1 DNA polymerase of S.cerevisiae: structural similarity with theE.coliDNA polymerase I-type enzymes, 10.1093/nar/19.4.955
  57. Ito Junetsu, Braithwaite Dan K., Yeast mitochondrial DNA polymerase is related to the family A DNA polymerases, 10.1093/nar/18.22.6716