E. Ruiz-pesini, M. T. Lott, V. Procaccio, J. C. Poole, M. C. Brandon et al., An enhanced MITOMAP with a global Nucleic Acids Research, p.431, 2007.
DOI : 10.1093/nar/gkl927
URL : https://academic.oup.com/nar/article-pdf/35/suppl_1/D823/3873213/gkl927.pdf

D. C. Wallace, Mitochondrial Diseases in Man and Mouse, Science, vol.283, issue.5407, pp.1482-1488, 1999.
DOI : 10.1126/science.283.5407.1482
URL : http://citeseerx.ist.psu.edu/viewdoc/summary?doi=10.1.1.322.3075

O. A. Kolesnikova, N. S. Entelis, H. Mireau, T. D. Fox, R. P. Martin et al., Suppression of Mutations in Mitochondrial DNA by tRNAs Imported from the Cytoplasm, Science, vol.289, issue.5486, pp.1931-1933, 2000.
DOI : 10.1126/science.289.5486.1931

B. Mahata, S. Mukherjee, S. Mishra, A. Bandyopadhyay, and S. Adhya, Functional Delivery of a Cytosolic tRNA into Mutant Mitochondria of Human Cells, Science, vol.314, issue.5798, pp.471-474, 2006.
DOI : 10.1126/science.1129754

G. Manfredi, J. Fu, J. Ojaimi, J. E. Sadlock, J. Q. Kwong et al., Rescue of a deficiency in ATP synthesis by transfer of MTATP6, a mitochondrial DNA-encoded gene, to the nucleus, Nature Genetics, vol.30, issue.4, pp.394-399, 2002.
DOI : 10.1038/ng851

O. Z. Karicheva, O. A. Kolesnikova, T. Schirtz, M. Y. Vysokikh, A. M. Mager-heckel et al., Correction of the consequences of mitochondrial 3243A>G mutation in the MT-TL1 gene causing the MELAS syndrome by tRNA import into mitochondria, Nucleic Acids Research, vol.39, issue.18, pp.8173-8186, 2011.
DOI : 10.1093/nar/gkr546

G. Wang, E. Shimada, J. Zhang, J. S. Hong, G. M. Smith et al., Correcting human mitochondrial mutations with targeted RNA import, Proc. Natl Acad. Sci. USA, pp.4840-4845, 2012.
DOI : 10.1093/nar/gkr580
URL : http://www.pnas.org/content/109/13/4840.full.pdf

S. R. Bacman, S. L. Williams, S. Garcia, and C. T. Moraes, Organ-specific shifts in mtDNA heteroplasmy following systemic delivery of a mitochondria-targeted restriction endonuclease, Gene Therapy, vol.21, issue.6, pp.713-720, 2010.
DOI : 10.1093/hmg/ddi307

S. R. Bacman, S. L. Williams, D. Hernandez, and C. T. Moraes, Modulating mtDNA heteroplasmy by mitochondria-targeted restriction endonucleases in a ???differential multiple cleavage-site??? model, Gene Therapy, vol.72, pp.1309-1318, 2007.
DOI : 10.2174/1566523043346200

M. Minczuk, M. A. Papworth, J. C. Miller, M. P. Murphy, and A. Klug, Development of a single-chain, quasi-dimeric zinc-finger nuclease for the selective degradation of mutated human mitochondrial DNA, Nucleic Acids Research, vol.36, issue.12, pp.3926-3938, 2008.
DOI : 10.1093/nar/gkn313

R. W. Taylor, P. F. Chinnery, D. M. Turnbull, and R. N. Lightowlers, Selective inhibition of mutant human mitochondrial DNA replication in vitro by peptide nucleic acids, Nature Genetics, vol.290, issue.2, pp.212-215, 1997.
DOI : 10.1002/ajmg.1320570334

P. F. Chinnery, R. W. Taylor, K. Diekert, R. Lill, D. M. Turnbull et al., Peptide nucleic acid delivery to human mitochondria, Gene Therapy, vol.6, issue.12, pp.1919-1928, 1999.
DOI : 10.1038/sj.gt.3301061
URL : http://www.nature.com/gt/journal/v6/n12/pdf/3301061a.pdf

A. Muratovska, R. N. Lightowlers, R. W. Taylor, D. M. Turnbull, R. A. Smith et al., Targeting peptide nucleic acid (PNA) oligomers to mitochondria within cells by conjugation to lipophilic cations: implications for mitochondrial DNA replication, expression and disease, Nucleic Acids Research, vol.29, issue.9, pp.1852-1863, 2001.
DOI : 10.1093/nar/29.9.1852
URL : https://www.ncbi.nlm.nih.gov/pmc/articles/PMC37250/pdf

P. M. Smith and R. N. Lightowlers, Altering the balance between healthy and mutated mitochondrial DNA, Journal of Inherited Metabolic Disease, vol.9, issue.2, pp.309-313, 2010.
DOI : 10.1093/brain/awl282

T. Salinas, A. M. Duchene, and L. Marechal-drouard, Recent advances in tRNA mitochondrial import, Trends in Biochemical Sciences, vol.33, issue.7, pp.320-329, 2008.
DOI : 10.1016/j.tibs.2008.04.010
URL : https://hal.archives-ouvertes.fr/hal-00309524

A. Schneider, Mitochondrial tRNA Import and Its Consequences for Mitochondrial Translation, Annual Review of Biochemistry, vol.80, issue.1, pp.1033-1053, 2011.
DOI : 10.1146/annurev-biochem-060109-092838

F. Sieber, A. M. Duchene, and L. Marechal-drouard, Mitochondrial RNA Import, Int. Rev. Cell. Mol. Biol, vol.287, pp.145-190, 2011.
DOI : 10.1016/B978-0-12-386043-9.00004-9

T. R. Mercer, S. Neph, M. E. Dinger, J. Crawford, M. A. Smith et al., The Human Mitochondrial Transcriptome, Cell, vol.146, issue.4, pp.645-658, 2011.
DOI : 10.1016/j.cell.2011.06.051
URL : http://doi.org/10.1016/j.cell.2011.06.051

B. T. Kren, P. Y. Wong, A. Sarver, X. Zhang, Y. Zeng et al., MicroRNAs identified in highly purified liver-derived mitochondria may play a role in apoptosis, RNA Biology, vol.6, issue.1, pp.65-72, 2009.
DOI : 10.4161/rna.6.1.7534

E. Barrey, G. Saint-auret, B. Bonnamy, D. Damas, O. Boyer et al., Pre-microRNA and Mature microRNA in Human Mitochondria, PLoS ONE, vol.34, issue.9, p.20220, 2011.
DOI : 10.1371/journal.pone.0020220.s009
URL : https://hal.archives-ouvertes.fr/inserm-00598509

S. Bandiera, S. Ruberg, M. Girard, N. Cagnard, S. Hanein et al., Nuclear Outsourcing of RNA Interference Components to Human Mitochondria, PLoS ONE, vol.10, issue.Pt 4, p.20746, 2011.
DOI : 10.1371/journal.pone.0020746.s013

O. A. Kolesnikova, N. S. Entelis, C. Jacquin-becker, F. Goltzene, Z. M. Chrzanowska-lightowlers et al., Nuclear DNA-encoded tRNAs targeted into mitochondria can rescue a mitochondrial DNA mutation associated with the MERRF syndrome in cultured human cells, Human Molecular Genetics, vol.13, issue.20, pp.2519-2534, 2004.
DOI : 10.1093/hmg/ddh267
URL : https://hal.archives-ouvertes.fr/hal-00153771

M. A. Rubio, J. J. Rinehart, B. Krett, S. Duvezin-caubet, A. S. Reichert et al., Mammalian mitochondria have the innate ability to import tRNAs by a mechanism distinct from protein import, Proc. Natl Acad. Sci. USA, pp.9186-9191, 2008.
DOI : 10.1016/0003-2697(87)90021-2

R. S. Puranam and G. Attardi, The RNase P Associated with HeLa Cell Mitochondria Contains an Essential RNA Component Identical in Sequence to That of the Nuclear RNase P, Molecular and Cellular Biology, vol.21, issue.2, pp.548-561, 2001.
DOI : 10.1128/MCB.21.2.548-561.2001

G. Wang, H. W. Chen, Y. Oktay, J. Zhang, E. L. Allen et al., PNPASE Regulates RNA Import into Mitochondria, Cell, vol.142, issue.3, pp.456-467, 2010.
DOI : 10.1016/j.cell.2010.06.035
URL : http://doi.org/10.1016/j.cell.2010.06.035

N. S. Entelis, O. A. Kolesnikova, S. Dogan, R. P. Martin, and I. A. Tarassov, 5 S rRNA and tRNA Import into Human Mitochondria, Journal of Biological Chemistry, vol.299, issue.49, pp.45642-45653, 2001.
DOI : 10.1016/0022-2836(87)90633-4
URL : https://hal.archives-ouvertes.fr/hal-00153774

P. J. Magalhaes, A. L. Andreu, and E. A. Schon, Evidence for the Presence of 5S rRNA in Mammalian Mitochondria, Molecular Biology of the Cell, vol.9, issue.9, pp.2375-2382, 1998.
DOI : 10.1091/mbc.9.9.2375

S. Yoshionari, T. Koike, T. Yokogawa, K. Nishikawa, T. Ueda et al., Existence of nuclear-encoded 5S-rRNA in bovine mitochondria, FEBS Letters, vol.11, issue.2, pp.137-142, 1994.
DOI : 10.1093/oxfordjournals.jbchem.a123673

A. Smirnov, C. Comte, A. M. Mager-heckel, V. Addis, I. A. Krasheninnikov et al., Mitochondrial Enzyme Rhodanese Is Essential for 5 S Ribosomal RNA Import into Human Mitochondria, Journal of Biological Chemistry, vol.68, issue.40, pp.30792-30803, 2010.
DOI : 10.1016/S0092-8674(01)00546-3
URL : http://www.jbc.org/content/285/40/30792.full.pdf

A. Smirnov, N. Entelis, R. P. Martin, and I. Tarassov, Biological significance of 5S rRNA import into human mitochondria: role of ribosomal protein MRP-L18, Genes & Development, vol.25, issue.12, pp.1289-1305, 2011.
DOI : 10.1101/gad.624711

A. Smirnov, I. Tarassov, A. M. Mager-heckel, M. Letzelter, R. P. Martin et al., Two distinct structural elements of 5S rRNA are needed for its import into human mitochondria, RNA, vol.14, issue.4, pp.749-759, 2008.
DOI : 10.1261/rna.952208

O. Kolesnikova, H. Kazakova, C. Comte, S. Steinberg, P. Kamenski et al., Selection of RNA aptamers imported into yeast and human mitochondria, RNA, vol.16, issue.5, pp.926-941, 2010.
DOI : 10.1261/rna.1914110

M. P. King and G. Attardi, Human cells lacking mtDNA: repopulation with exogenous mitochondria by complementation, Science, vol.246, issue.4929, pp.500-503, 1989.
DOI : 10.1126/science.2814477

A. M. Mager-heckel, N. Entelis, I. Brandina, P. Kamenski, I. A. Krasheninnikov et al., The Analysis of tRNA Import Into Mammalian Mitochondria, Methods Mol. Biol, vol.372, pp.235-253, 2007.
DOI : 10.1007/978-1-59745-365-3_17

P. A. Srere, Spectral evidence for complex formation between oxaloacetate and citrate-condensing enzyme, Biochimica et Biophysica Acta (BBA) - Enzymology and Biological Oxidation, vol.99, issue.1, pp.197-200, 1965.
DOI : 10.1016/S0926-6593(65)80028-5

D. Kern, A. Dietrich, F. Fasiolo, M. Renaud, R. Giege et al., The yeast aminoacyl-tRNA synthetases, Biochimie, vol.59, issue.5-6, pp.453-462, 1977.
DOI : 10.1016/S0300-9084(77)80050-3

N. S. Entelis, I. A. Krasheninnikov, R. P. Martin, and I. A. Tarassov, : possible roles of aminoacylation and modified nucleosides in subcellular partitioning, FEBS Letters, vol.85, issue.1, pp.38-42, 1996.
DOI : 10.1038/344787a0

E. Kun, E. Kirsten, and W. N. Piper, [14] Stabilization of mitochondrial functions with digitonin, Methods Enzymol, vol.55, pp.115-118, 1979.
DOI : 10.1016/0076-6879(79)55016-2

T. W. Wong and D. A. Clayton, In vitro replication of human mitochondrial DNA: Accurate initiation at the origin of light-strand synthesis, Cell, vol.42, issue.3, pp.951-958, 1985.
DOI : 10.1016/0092-8674(85)90291-0

T. W. Wong and D. A. Clayton, Isolation and characterization of a DNA primase from human mitochondria, J. Biol. Chem, vol.260, pp.11530-11535, 1985.

C. Kohrer, L. Xie, S. Kellerer, U. Varshney, and U. L. Rajbhandary, Import of amber and ochre suppressor tRNAs into mammalian cells: A general approach to site-specific insertion of amino acid analogues into proteins, Proc. Natl Acad. Sci. USA, pp.14310-14315, 2001.
DOI : 10.1128/jb.177.10.2858-2862.1995

A. Reyes, T. Yasukawa, and I. J. Holt, Analysis of Replicating Mitochondrial DNA by Two-Dimensional Agarose Gel Electrophoresis, Methods Mol. Biol, vol.372, pp.219-232, 2007.
DOI : 10.1007/978-1-59745-365-3_16

A. K. Hyvarinen, J. L. Pohjoismaki, A. Reyes, S. Wanrooij, T. Yasukawa et al., The mitochondrial transcription termination factor mTERF modulates replication pausing in human mitochondrial DNA, Nucleic Acids Research, vol.35, issue.19, pp.6458-6474, 2007.
DOI : 10.1093/nar/gkm676

A. Reyes, T. Yasukawa, T. J. Cluett, and I. J. Holt, Analysis of Mitochondrial DNA by Two-Dimensional Agarose Gel Electrophoresis, Methods Mol. Biol, vol.554, pp.15-35, 2009.
DOI : 10.1007/978-1-59745-521-3_2

N. R. Markham and M. Zuker, DINAMelt web server for nucleic acid melting prediction, Nucleic Acids Research, vol.33, issue.Web Server, pp.577-581, 2005.
DOI : 10.1093/nar/gki591
URL : https://academic.oup.com/nar/article-pdf/33/suppl_2/W577/7623865/gki591.pdf

N. R. Markham and M. Zuker, UNAFold, Methods Mol. Biol, vol.453, pp.3-31, 2008.
DOI : 10.1007/978-1-60327-429-6_1

M. Szymanski, M. Z. Barciszewska, V. A. Erdmann, and J. Barciszewski, 5S Ribosomal RNA Database, Nucleic Acids Research, vol.30, issue.1, pp.176-178, 2002.
DOI : 10.1093/nar/30.1.176
URL : https://www.ncbi.nlm.nih.gov/pmc/articles/PMC99124/pdf

M. Szymanski, M. Z. Barciszewska, V. A. Erdmann, and J. Barciszewski, 5 S rRNA: structure and interactions, Biochemical Journal, vol.371, issue.3, pp.641-651, 2003.
DOI : 10.1042/bj20020872

N. Sugimoto, S. Nakano, M. Katoh, A. Matsumura, H. Nakamuta et al., Thermodynamic Parameters To Predict Stability of RNA/DNA Hybrid Duplexes, Biochemistry, vol.34, issue.35, pp.11211-11216, 1995.
DOI : 10.1021/bi00035a029

B. Bornstein, J. A. Mas, M. A. Fernandez-moreno, Y. Campos, M. A. Martin et al., The A8296G mtDNA mutation associated with several mitochondrial diseases does not cause mitochondrial dysfunction in cybrid cell lines, Human Mutation, vol.89, issue.3, pp.234-239, 2002.
DOI : 10.1073/pnas.89.23.11164

S. Dimauro and E. A. Schon, Mitochondrial DNA mutations in human disease, American Journal of Medical Genetics, vol.38, issue.1, pp.18-26, 2001.
DOI : 10.1212/WNL.38.8.1339

I. J. Holt, H. E. Lorimer, and H. T. Jacobs, Coupled Leading- and Lagging-Strand Synthesis of Mammalian Mitochondrial DNA, Cell, vol.100, issue.5, pp.515-524, 2000.
DOI : 10.1016/S0092-8674(00)80688-1
URL : http://doi.org/10.1016/s0092-8674(00)80688-1

M. Bowmaker, M. Y. Yang, T. Yasukawa, A. Reyes, H. T. Jacobs et al., Mammalian Mitochondrial DNA Replicates Bidirectionally from an Initiation Zone, Journal of Biological Chemistry, vol.82, issue.51, pp.50961-50969, 2003.
DOI : 10.1093/embo-reports/kve233

A. K. Hyvarinen, J. L. Pohjoismaki, I. J. Holt, and H. T. Jacobs, Overexpression of MTERFD1 or MTERFD3 impairs the completion of mitochondrial DNA replication, Molecular Biology Reports, vol.25, issue.2, pp.1321-1328, 2011.
DOI : 10.1038/sj.emboj.7601392

T. Yasukawa, A. Reyes, T. J. Cluett, M. Y. Yang, M. Bowmaker et al., Replication of vertebrate mitochondrial DNA entails transient ribonucleotide incorporation throughout the lagging strand, The EMBO Journal, vol.21, issue.22, pp.5358-5371, 2006.
DOI : 10.1038/ni919

J. L. Pohjoismaki, J. B. Holmes, S. R. Wood, M. Y. Yang, T. Yasukawa et al., Mammalian Mitochondrial DNA Replication Intermediates Are Essentially Duplex but Contain Extensive Tracts of RNA/DNA Hybrid, Journal of Molecular Biology, vol.397, issue.5, pp.1144-1155, 2010.
DOI : 10.1016/j.jmb.2010.02.029

S. Wanrooij, S. Goffart, J. L. Pohjoismaki, T. Yasukawa, and J. N. Spelbrink, Expression of catalytic mutants of the mtDNA helicase Twinkle and polymerase POLG causes distinct replication stalling phenotypes, Nucleic Acids Research, vol.35, issue.10, pp.3238-3251, 2007.
DOI : 10.1093/nar/gkm215

T. A. Brown, C. Cecconi, A. N. Tkachuk, C. Bustamante, and D. A. Clayton, Replication of mitochondrial DNA occurs by strand displacement with alternative light-strand origins, not via a strand-coupled mechanism, Genes & Development, vol.19, issue.20, pp.2466-2476, 2005.
DOI : 10.1101/gad.1352105

P. M. Smith, G. F. Ross, R. W. Taylor, D. M. Turnbull, and R. N. Lightowlers, Strategies for treating disorders of the mitochondrial genome, Biochimica et Biophysica Acta (BBA) - Bioenergetics, vol.1659, issue.2-3, pp.1659-232, 2004.
DOI : 10.1016/j.bbabio.2004.09.003

I. J. Holt, Mitochondrial DNA replication and repair: all a flap, Trends in Biochemical Sciences, vol.34, issue.7, pp.358-365, 2009.
DOI : 10.1016/j.tibs.2009.03.007

D. A. Clayton, Mitochondrial DNA Replication: What We Know, IUBMB Life (International Union of Biochemistry and Molecular Biology: Life), vol.55, issue.4-5, pp.213-217, 2003.
DOI : 10.1080/1521654031000134824

J. L. Pohjoismaki and S. Goffart, Of circles, forks and humanity: Topological organisation and replication of mammalian mitochondrial DNA, BioEssays, vol.44, issue.4, pp.290-299, 2011.
DOI : 10.1146/annurev-genet-102108-134841

D. A. Clayton, Replication of animal mitochondrial DNA, Cell, vol.28, issue.4, pp.693-705, 1982.
DOI : 10.1016/0092-8674(82)90049-6

C. T. Moraes, L. Kenyon, and H. Hao, Mechanisms of Human Mitochondrial DNA Maintenance: The Determining Role of Primary Sequence and Length over Function, Molecular Biology of the Cell, vol.10, issue.10, pp.3345-3356, 1999.
DOI : 10.1091/mbc.10.10.3345

F. Diaz, M. P. Bayona-bafaluy, M. Rana, M. Mora, H. Hao et al., Human mitochondrial DNA with large deletions repopulates organelles faster than full-length genomes under relaxed copy number control, Nucleic Acids Research, vol.30, issue.21, pp.4626-4633, 2002.
DOI : 10.1093/nar/gkf602
URL : https://www.ncbi.nlm.nih.gov/pmc/articles/PMC135822/pdf

B. Bornstein, J. A. Mas, C. Patrono, M. A. Fernandez-moreno, E. Gonzalez-vioque et al., gene, Biochemical Journal, vol.387, issue.3, pp.773-778, 2005.
DOI : 10.1042/BJ20040949