Abstract
Mitochondria are key players in cellular metabolism and energy production. They have their own genetic system. The mitochondrial genome is small and contains a limited number of genes, but its expression is complex and essential for survival. Organelle DNA mutations or rearrangements cause incurable neurodegenerative diseases in humans or cytoplasmic male sterility in plants. Manipulating mitochondrial genetics is thus of particular relevance. Conventional transformation of these organelles was only achieved in a couple of unicellular organisms, but the importance of the issue stimulated the development of a wealth of alternative strategies. These include a variety of approaches aiming to transfer DNA into mitochondria and maintain the transfected genetic information, although the problem of selection markers for mitochondrial transformants remains open in most organisms. Conversely, nuclear expression and mitochondrial trafficking of proteins or RNAs through different targeting and shuttling systems have been widely developed to circumvent the need for regular transformation. A number of these methodologies were reported to be successful, for instance to rescue pathogenic mutations in the mitochondrial genome, others are promising. The challenge in the field is currently to define consensus biotechnological tools.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Alexeyev MF, Venediktova N, Pastukh V, Shokolenko I, Bonilla G, Wilson GL (2008) Selective elimination of mutant mitochondrial genomes as therapeutic strategy for the treatment of NARP and MILS syndromes. Gene Ther 15:516–523
Bacman SR, Williams SL, Hernandez D, Moraes CT (2007) Modulating mtDNA heteroplasmy by mitochondria-targeted restriction endonucleases in a ‘differential multiple cleavage-site’ model. Gene Ther 14:1309–1318
Bacman SR, Williams SL, Garcia S, Moraes CT (2010) Organ-specific shifts in mtDNA heteroplasmy following systemic delivery of a mitochondria-targeted restriction endonuclease. Gene Ther 17:713–720
Bakker BM, Overkamp KM, van Maris AJ, Kotter P, Luttik MA, van Dijken JP, Pronk JT (2001) Stoichiometry and compartmentation of NADH metabolism in Saccharomyces cerevisiae. FEMS Microbiol Rev 25:15–37
Baroux C, Blanvillain R, Gallois P (2001a) Paternally inherited transgenes are down-regulated but retain low activity during early embryogenesis in Arabidopsis. FEBS Lett 509:11–16
Baroux C, Blanvillain R, Moore IR, Gallois P (2001b) Transactivation of BARNASE under the AtLTP1 promoter affects the basal pole of the embryo and shoot development of the adult plant in Arabidopsis. Plant J 28:503–515
Bayona-Bafaluy MP, Blits B, Battersby BJ, Shoubridge EA, Moraes CT (2005) Rapid directional shift of mitochondrial DNA heteroplasmy in animal tissues by a mitochondrially targeted restriction endonuclease. Proc Natl Acad Sci U S A 102:14392–14397
Bellaoui M, Martin-Canadell A, Pelletier G, Budar F (1998) Low-copy-number molecules are produced by recombination, actively maintained and can be amplified in the mitochondrial genome of Brassicaceae: relationship to reversion of the male sterile phenotype in some cybrids. Mol Gen Genet 257:177–185
Bennoun P, Delosme M, Kuck U (1991) Mitochondrial genetics of Chlamydomonas reinhardtii: resistance mutations marking the cytochrome b gene. Genetics 127:335–343
Ber R, Stauver MG, Shay JW (1984) Use of isolated mitochondria to transfer chloramphenicol resistance in hamster cells. Isr J Med Sci 20:244–248
Bhattacharyya SN, Chatterjee S, Goswami S, Tripathi G, Dey SN, Adhya S (2003) “Ping-pong” interactions between mitochondrial tRNA import receptors within a multiprotein complex. Mol Cell Biol 23:5217–5224
Blanc H, Adams CW, Wallace DC (1981) Different nucleotide changes in the large rRNA gene of the mitochondrial DNA confer chloramphenicol resistance on two human cell lines. Nucleic Acids Res 9:5785–5795
Boddapati SV, Tongcharoensirikul P, Hanson RN, D’Souza GG, Torchilin VP, Weissig V (2005) Mitochondriotropic liposomes. J Liposome Res 15:49–58
Boesch P, Ibrahim N, Paulus F, Cosset A, Tarasenko V, Dietrich A (2009) Plant mitochondria possess a short-patch base excision DNA repair pathway. Nucleic Acids Res 37:5690–5700
Boesch P, Ibrahim N, Dietrich A, Lightowlers RN (2010) Membrane association of mitochondrial DNA facilitates base excision repair in mammalian mitochondria. Nucleic Acids Res 38:1478–1488
Bokori-Brown M, Holt IJ (2006) Expression of algal nuclear ATP synthase subunit 6 in human cells results in protein targeting to mitochondria but no assembly into ATP synthase. Rejuvenation Res 9:455–469
Bolle N, Kempken F (2006) Mono- and dicotyledonous plant-specific RNA editing sites are correctly edited in both in organello systems. FEBS Lett 580:4443–4448
Bolle N, Hinrichsen I, Kempken F (2007) Plastid mRNAs are neither spliced nor edited in maize and cauliflower mitochondrial in organello systems. RNA 13:2061–2065
Bonnefoy N, Fox TD (2000) In vivo analysis of mutated initiation codons in the mitochondrial COX2 gene of Saccharomyces cerevisiae fused to the reporter gene ARG8m reveals lack of downstream reinitiation. Mol Gen Genet 262:1036–1046
Bonnefoy N, Fox TD (2007) Directed alteration of Saccharomyces cerevisiae mitochondrial DNA by biolistic transformation and homologous recombination. Methods Mol Biol 372:153–166
Bonnefoy N, Bsat N, Fox TD (2001) Mitochondrial translation of Saccharomyces cerevisiae COX2 mRNA is controlled by the nucleotide sequence specifying the pre-Cox2p leader peptide. Mol Cell Biol 21:2359–2372
Bonnefoy N, Remacle C, Fox TD (2007) Genetic transformation of Saccharomyces cerevisiae and Chlamydomonas reinhardtii mitochondria. Methods Cell Biol 80:525–548
Bonnet C, Kaltimbacher V, Ellouze S, Augustin S, Benit P, Forster V, Rustin P, Sahel JA, Corral-Debrinski M (2007) Allotopic mRNA localization to the mitochondrial surface rescues respiratory chain defects in fibroblasts harboring mitochondrial DNA mutations affecting complex I or v subunits. Rejuvenation Res 10:127–144
Bonnet C, Augustin S, Ellouze S, Benit P, Bouaita A, Rustin P, Sahel JA, Corral-Debrinski M (2008) The optimized allotopic expression of ND1 or ND4 genes restores respiratory chain complex I activity in fibroblasts harboring mutations in these genes. Biochim Biophys Acta 1783:1707–1717
Bowmaker M, Yang MY, Yasukawa T, Reyes A, Jacobs HT, Huberman JA, Holt IJ (2003) Mammalian mitochondrial DNA replicates bidirectionally from an initiation zone. J Biol Chem 278:50961–50969
Boynton JE, Gillham NW, Harris EH, Hosler JP, Johnson AM, Jones AR, Randolph-Anderson BL, Robertson D, Klein TM, Shark KB, Sanford JC (1988) Chloroplast transformation in Chlamydomonas with high velocity microprojectiles. Science 240:1534–1538
Brandina I, Smirnov A, Kolesnikova O, Entelis N, Krasheninnikov IA, Martin RP, Tarassov I (2007) tRNA import into yeast mitochondria is regulated by the ubiquitin-proteasome system. FEBS Lett 581:4248–4254
Chang DD, Clayton DA (1987) A mammalian mitochondrial RNA processing activity contains nucleus-encoded RNA. Science 235:1178–1184
Chang DD, Clayton DA (1989) Mouse RNAase MRP RNA is encoded by a nuclear gene and contains a decamer sequence complementary to a conserved region of mitochondrial RNA substrate. Cell 56:131–139
Chase CD (2007) Cytoplasmic male sterility: a window to the world of plant mitochondrial-nuclear interactions. Trends Genet 23:81–90
Chatterjee S, Home P, Mukherjee S, Mahata B, Goswami S, Dhar G, Adhya S (2006) An RNA-binding respiratory component mediates import of type II tRNAs into Leishmania mitochondria. J Biol Chem 281:25270–25277
Chaumont F, Bernier B, Buxant R, Williams ME, Levings CS 3rd, Boutry M (1995) Targeting the maize T-urf13 product into tobacco mitochondria confers methomyl sensitivity to mitochondrial respiration. Proc Natl Acad Sci U S A 92:1167–1171
Chiu N, Chiu AO, Suyama Y (1974) Three isoaccepting forms of leucyl transfer RNA in mitochondria. J Mol Biol 82:441–457
Choury D, Araya A (2006) RNA editing site recognition in heterologous plant mitochondria. Curr Genet 50:405–416
Choury D, Farre JC, Jordana X, Araya A (2004) Different patterns in the recognition of editing sites in plant mitochondria. Nucleic Acids Res 32:6397–6406
Choury D, Farre JC, Jordana X, Araya A (2005) Gene expression studies in isolated mitochondria: Solanum tuberosum rps10 is recognized by cognate potato but not by the transcription, splicing and editing machinery of wheat mitochondria. Nucleic Acids Res 33:7058–7065
Clark MA, Shay JW (1982) Mitochondrial transformation of mammalian cells. Nature 295:605–607
Clayton DA (2003) Mitochondrial DNA replication: what we know. IUBMB Life 55:213–217
Colin M, Dorthu MP, Duby F, Remacle C, Dinant M, Wolwertz MR, Duyckaerts C, Sluse F, Matagne RF (1995) Mutations affecting the mitochondrial genes encoding the cytochrome oxidase subunit I and apocytochrome b of Chlamydomonas reinhardtii. Mol Gen Genet 249:179–184
Collombet JM, Wheeler VC, Vogel F, Coutelle C (1997) Introduction of plasmid DNA into isolated mitochondria by electroporation. A novel approach toward gene correction for mitochondrial disorders. J Biol Chem 272:5342–5347
Comte C, Tonin Y, Heckel-Mager AM, Boucheham A, Smirnov A, Aure K, Lombes A, Martin RP, Entelis N, Tarassov I (2012) Mitochondrial targeting of recombinant RNAs modulates the level of a heteroplasmic mutation in human mitochondrial DNA associated with Kearns Sayre Syndrome. Nucleic Acids Res. doi:10.1093/nar/gks1965
Cristodero M, Seebeck T, Schneider A (2010) Mitochondrial translation is essential in bloodstream forms of Trypanosoma brucei. Mol Microbiol 78:757–769
Cudd A, Nicolau C (1985) Intracellular fate of liposome-encapsulated DNA in mouse liver. Analysis using electron microscope autoradiography and subcellular fractionation. Biochim Biophys Acta 845:477–491
Cudd A, Nicolau C (1986) Interaction of intravenously injected liposomes with mouse liver mitochondria. A fluorescence and electron microscopy study. Biochim Biophys Acta 860:201–214
Cudd A, Labbe H, Gervais M, Nicolau C (1984) Liposomes injected intravenously into mice associate with liver mitochondria. Biochim Biophys Acta 774:169–180
Cwerman-Thibault H, Sahel JA, Corral-Debrinski M (2011) Mitochondrial medicine: to a new era of gene therapy for mitochondrial DNA mutations. J Inherit Metab Dis 34:327–344
D’Souza GG, Rammohan R, Cheng SM, Torchilin VP, Weissig V (2003) DQAsome-mediated delivery of plasmid DNA toward mitochondria in living cells. J Control Release 92:189–197
D’Souza GG, Boddapati SV, Weissig V (2007) Gene therapy of the other genome: the challenges of treating mitochondrial DNA defects. Pharm Res 24:228–238
Dassa EP, Dufour E, Goncalves S, Paupe V, Hakkaart GA, Jacobs HT, Rustin P (2009) Expression of the alternative oxidase complements cytochrome c oxidase deficiency in human cells. EMBO Mol Med 1:30–36
Dewey RE, Levings CS 3rd, Timothy DH (1986) Novel recombinations in the maize mitochondrial genome produce a unique transcriptional unit in the Texas male-sterile cytoplasm. Cell 44:439–449
di Rago JP, Colson AM (1988) Molecular basis for resistance to antimycin and diuron, Q-cycle inhibitors acting at the Qi site in the mitochondrial ubiquinol-cytochrome c reductase in Saccharomyces cerevisiae. J Biol Chem 263:12564–12570
di Rago JP, Coppee JY, Colson AM (1989) Molecular basis for resistance to myxothiazol, mucidin (strobilurin A), and stigmatellin. Cytochrome b inhibitors acting at the center o of the mitochondrial ubiquinol-cytochrome c reductase in Saccharomyces cerevisiae. J Biol Chem 264:14543–14548
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
Dörner M, Altmann M, Paabo S, Morl M (2001) Evidence for import of a lysyl-tRNA into marsupial mitochondria. Mol Biol Cell 12:2688–2698
Dorthu MP, Remy S, Michel-Wolwertz MR, Colleaux L, Breyer D, Beckers MC, Englebert S, Duyckaerts C, Sluse FE, Matagne RF (1992) Biochemical, genetic and molecular characterization of new respiratory-deficient mutants in Chlamydomonas reinhardtii. Plant Mol Biol 18:759–772
Dudek J, Rehling P, van der Laan M (2012) Mitochondrial protein import: common principles and physiological networks. Biochim Biophys Acta. doi:10.1016/j.bbamcr.2012.1005.1028
Dujon B (1981) Mitochondrial genetics and functions. In: Strathern JN, Jones EW, Broach JR (eds) The molecular biology of the yeast Saccharomyces: life cycle and inheritance. Cold Spring Harbor Laboratory, Cold Spring Harbor, pp 505–635
Dunn DA, Pinkert CA (2012) Nuclear expression of a mitochondrial DNA gene: mitochondrial targeting of allotopically expressed mutant ATP6 in transgenic mice. J Biomed Biotechnol 2012:541245
Duroc Y, Gaillard C, Hiard S, Tinchant C, Berthomé R, Pelletier R, Budar F (2006) Nuclear expression of a cytoplasmic male sterility gene modifies mitochondrial morphology in yeast and plant cells. Plant Sci 170:755–767
Ellouze S, Augustin S, Bouaita A, Bonnet C, Simonutti M, Forster V, Picaud S, Sahel JA, Corral-Debrinski M (2008) Optimized allotopic expression of the human mitochondrial ND4 prevents blindness in a rat model of mitochondrial dysfunction. Am J Hum Genet 83:373–387
Elson JL, Lightowlers RN (2006) Mitochondrial DNA clonality in the dock: can surveillance swing the case? Trends Genet 22:603–607
Entelis NS, Kolesnikova OA, Dogan S, Martin RP, Tarassov IA (2001) 5S rRNA and tRNA import into human mitochondria. Comparison of in vitro requirements. J Biol Chem 276:45642–45653
Entelis N, Brandina I, Kamenski P, Krasheninnikov IA, Martin RP, Tarassov I (2006) A glycolytic enzyme, enolase, is recruited as a cofactor of tRNA targeting toward mitochondria in Saccharomyces cerevisiae. Genes Dev 20:1609–1620
Estevez AM, Thiemann OH, Alfonzo JD, Simpson L (1999) T7 RNA polymerase-driven transcription in mitochondria of Leishmania tarentolae and Trypanosoma brucei. Mol Biochem Parasitol 103:251–259
Eudes F, Chugh A (2008) Nanocarrier-based plant transfection and transduction. Patent, Publication number WO/2008/148223, Application number PCT/CA2008/001112, Country CA
Falkenberg M, Larsson NG, Gustafsson CM (2007) DNA replication and transcription in mammalian mitochondria. Annu Rev Biochem 76:679–699
Fan W, Lin CS, Potluri P, Procaccio V, Wallace DC (2012) mtDNA lineage analysis of mouse L-cell lines reveals the accumulation of multiple mtDNA mutants and intermolecular recombination. Genes Dev 26:384–394
Farré JC, Araya A (2001) Gene expression in isolated plant mitochondria: high fidelity of transcription, splicing and editing of a transgene product in electroporated organelles. Nucleic Acids Res 29:2484–2491
Farré JC, Araya A (2002) RNA splicing in higher plant mitochondria: determination of functional elements in group II intron from a chimeric cox II gene in electroporated wheat mitochondria. Plant J 29:203–213
Farré JC, Leon G, Jordana X, Araya A (2001) cis-Recognition elements in plant mitochondrion RNA editing. Mol Cell Biol 21:6731–6737
Flierl A, Jackson C, Cottrell B, Murdock D, Seibel P, Wallace DC (2003) Targeted delivery of DNA to the mitochondrial compartment via import sequence-conjugated peptide nucleic acid. Mol Ther 7:550–557
Fox TD, Sanford JC, McMullin TW (1988) Plasmids can stably transform yeast mitochondria lacking endogenous mtDNA. Proc Natl Acad Sci USA 85:7288–7292
Frei U, Peiretti EG, Wenzel G (2004) Significance of cytoplasmic DNA in plant breeding. In: Janick J (ed) Plant breeding reviews. Wiley, Hoboken, pp 175–210
Goldschmidt-Clermont M (1991) Transgenic expression of aminoglycoside adenine transferase in the chloroplast: a selectable marker of site-directed transformation of Chlamydomonas. Nucleic Acids Res 19:4083–4089
Goswami S, Dhar G, Mukherjee S, Mahata B, Chatterjee S, Home P, Adhya S (2006) A bifunctional tRNA import receptor from Leishmania mitochondria. Proc Natl Acad Sci U S A 103:8354–8359
Greaves LC, Reeve AK, Taylor RW, Turnbull DM (2012) Mitochondrial DNA and Disease. J Pathol 226:274–286
Guy J, Qi X, Pallotti F, Schon EA, Manfredi G, Carelli V, Martinuzzi A, Hauswirth WW, Lewin AS (2002) Rescue of a mitochondrial deficiency causing Leber Hereditary Optic Neuropathy. Ann Neurol 52:534–542
Hancock K, Hajduk SL (1990) The mitochondrial tRNAs of Trypanosoma brucei are nuclear-encoded. J Biol Chem 265:19208–19215
Handa H (2008) Linear plasmids in plant mitochondria: peaceful coexistences or malicious invasions? Mitochondrion 8:15–25
Hartley RW (1988) Barnase and barstar. Expression of its cloned inhibitor permits expression of a cloned ribonuclease. J Mol Biol 202:913–915
He S, Abad AR, Gelvin SB, Mackenzie SA (1996) A cytoplasmic male sterility-associated mitochondrial protein causes pollen disruption in transgenic tobacco. Proc Natl Acad Sci U S A 93:11763–11768
Hinrichsen I, Bolle N, Paun L, Kempken F (2009) RNA processing in plant mitochondria is independent of transcription. Plant Mol Biol 70:663–668
Holt IJ, Lorimer HE, Jacobs HT (2000) Coupled leading- and lagging-strand synthesis of mammalian mitochondrial DNA. Cell 100:515–524
Holzmann J, Frank P, Loffler E, Bennett KL, Gerner C, Rossmanith W (2008) RNase P without RNA: identification and functional reconstitution of the human mitochondrial tRNA processing enzyme. Cell 135:462–474
Horobin RW, Trapp S, Weissig V (2007) Mitochondriotropics: a review of their mode of action, and their applications for drug and DNA delivery to mammalian mitochondria. J Control Release 121:125–136
Howell N, Gilbert K (1988) Mutational analysis of the mouse mitochondrial cytochrome b gene. J Mol Biol 203:607–618
Hyrup B, Nielsen PE (1996) Peptide nucleic acids (PNA): synthesis, properties and potential applications. Bioorg Med Chem 4:5–23
Ibrahim N, Handa H, Cosset A, Koulintchenko M, Konstantinov Y, Lightowlers RN, Dietrich A, Weber-Lotfi F (2011) DNA delivery to mitochondria: sequence specificity and energy enhancement. Pharm Res 28:2871–2882
Inoki Y, Hakamata Y, Hamamoto T, Kinouchi T, Yamazaki S, Kagawa Y, Endo H (2000) Proteoliposomes colocalized with endogenous mitochondria in mouse fertilized egg. Biochem Biophys Res Commun 278:183–191
Iyer S, Thomas RR, Portell FR, Dunham LD, Quigley CK, Bennett JP, Jr (2009) Recombinant mitochondrial transcription factor A with N-terminal mitochondrial transduction domain increases respiration and mitochondrial gene expression. Mitochondrion 9:196–203
Iyer S, Bergquist K, Young K, Gnaiger E, Rao RR, Bennett JP, Jr (2012a) Mitochondrial gene therapy improves respiration, biogenesis, and transcription in G11778A Leber’s hereditary optic neuropathy and T8993G Leigh’s syndrome cells. Hum Gene Ther 23:647–657
Iyer S, Xiao E, Alsayegh K, Eroshenko N, Riggs MJ, Bennett JP, Jr. and Rao RR (2012b) Mitochondrial gene replacement in human pluripotent stem cell-derived neural progenitors. Gene Ther 19:469–475
Janicka S, Kuhn K, Le Ret M, Bonnard G, Imbault P, Augustyniak H, Gualberto JM (2012) A RAD52-like single-stranded DNA binding protein affects mitochondrial DNA repair by recombination. Plant J. doi:10.1111/j.1365-1313X.2012.05097.x
Jash S, Adhya S (2011) Suppression of reactive oxygen species in cells with multiple mitochondrial DNA deletions by exogenous protein-coding RNAs. Mitochondrion 11:607–614
Jash S, Chowdhury T, Adhya S (2012) Modulation of mitochondrial respiratory capacity by carrier-mediated transfer of RNA in vivo. Mitochondrion 12:262–270
Jenuth JP, Peterson AC, Fu K, Shoubridge EA (1996) Random genetic drift in the female germline explains the rapid segregation of mammalian mitochondrial DNA. Nat Genet 14:146–151
Johnston SA, Anziano PQ, Shark K, Sanford JC, Butow RA (1988) Mitochondrial transformation in yeast by bombardment with microprojectiles. Science 240:1538–1541
Kagawa Y, Hayashi JI (1997) Gene therapy of mitochondrial diseases using human cytoplasts. Gene Ther 4:6–10
Kagawa Y, Inoki Y, Endo H (2001) Gene therapy by mitochondrial transfer. Adv Drug Deliv Rev 49:107–119
Kaltimbacher V, Bonnet C, Lecoeuvre G, Forster V, Sahel JA, Corral-Debrinski M (2006) mRNA localization to the mitochondrial surface allows the efficient translocation inside the organelle of a nuclear recoded ATP6 protein. RNA 12:1408–1417
Karicheva OZ, Kolesnikova OA, Schirtz T, Vysokikh MY, Mager-Heckel AM, Lombes A, Boucheham A, Krasheninnikov IA, Martin RP, Entelis N, Tarassov I (2011) 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 Res 39:8173–8186
Katrangi E, D’Souza G, Boddapati SV, Kulawiec M, Singh KK, Bigger B, Weissig V (2007) Xenogenic transfer of isolated murine mitochondria into human rho0 cells can improve respiratory function. Rejuvenation Res 10:561–570
Keeney PM, Quigley CK, Dunham LD, Papageorge CM, Iyer S, Thomas RR, Schwarz KM, Trimmer PA, Khan SM, Portell FR, Bergquist KE, Bennett JP, Jr (2009) Mitochondrial gene therapy augments mitochondrial physiology in a Parkinson’s disease cell model. Hum Gene Ther 20:897–907
Kempken F, Bolle N, Forner J, Binder S (2007) Transcript end mapping and analysis of RNA editing in plant mitochondria. Methods Mol Biol 372:177–192
Khan SM, Bennett JP, Jr (2004) Development of mitochondrial gene replacement therapy. J Bioenerg Biomembr 36:387–393
Kim DH, Kang JG, Kim BD (2007) Isolation and characterization of the cytoplasmic male sterility-associated orf456 gene of chili pepper (Capsicum annuum L.). Plant Mol Biol 63:519–532
Kindle KL, Richards KL, Stern DB (1991) Engineering the chloroplast genome: techniques and capabilities for chloroplast transformation in Chlamydomonas reinhardtii. Proc Natl Acad Sci U S A 88:1721–1725
King MP, Attardi G (1988) Injection of mitochondria into human cells leads to a rapid replacement of the endogenous mitochondrial DNA. Cell 52:811–819
King MP, Attardi G (1989) Human cells lacking mtDNA: repopulation with exogenous mitochondria by complementation. Science 246:500–503
King MP, Koga Y, Davidson M, Schon EA (1992) Defects in mitochondrial protein synthesis and respiratory chain activity segregate with the tRNA(Leu(UUR)) mutation associated with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes. Mol Cell Biol 12:480–490
Kolesnikova OA, Entelis NS, Mireau H, Fox TD, Martin RP, Tarassov IA (2000) Suppression of mutations in mitochondrial DNA by tRNAs imported from the cytoplasm. Science 289:1931–1933
Kolesnikova OA, Entelis NS, Jacquin-Becker C, Goltzene F, Chrzanowska-Lightowlers ZM, Lightowlers RN, Martin RP, Tarassov I (2004) Nuclear DNA-encoded tRNAs targeted into mitochondria can rescue a mitochondrial DNA mutation associated with the MERRF syndrome in cultured human cells. Hum Mol Genet 13:2519–2534
Kolesnikova O, Kazakova H, Comte C, Steinberg S, Kamenski P, Martin RP, Tarassov I, Entelis N (2010) Selection of RNA aptamers imported into yeast and human mitochondria. RNA 16:926–941
Koulintchenko M, Konstantinov Y, Dietrich A (2003) Plant mitochondria actively import DNA via the permeability transition pore complex. EMBO J 22:1245–1254
Koulintchenko M, Temperley RJ, Mason PA, Dietrich A, Lightowlers RN (2006) Natural competence of mammalian mitochondria allows the molecular investigation of mitochondrial gene expression. Hum Mol Genet 15:143–154
Kühn K, Gualberto JM (2012) Recombination in the stability, repair and evolution of the mitochondrial genome. Adv Bot Res 63:215–252
Kumar R, Maréchal-Drouard L, Akama K, Small I (1996) Striking differences in mitochondrial tRNA import between different plant species. Mol Gen Genet 252:404–411
Kumar P, Vasupalli N, Srinivasan R, Bhat SR (2012) An evolutionarily conserved mitochondrial orf108 is associated with cytoplasmic male sterility in different alloplasmic lines of Brassica juncea and induces male sterility in transgenic Arabidopsis thaliana. J Exp Bot 63:2921–2932
Lee DY, Clayton DA (1997) RNase mitochondrial RNA processing correctly cleaves a novel R loop at the mitochondrial DNA leading-strand origin of replication. Genes Dev 11:582–592
Lee DY, Clayton DA (1998) Initiation of mitochondrial DNA replication by transcription and R-loop processing. J Biol Chem 273:30614–30621
Lee M, Choi JS, Choi MJ, Pak YK, Rhee BD, Ko KS (2007) DNA delivery to the mitochondria sites using mitochondrial leader peptide conjugated polyethylenimine. J Drug Target 15:115–122
Li XQ, Chetrit P, Mathieu C, Vedel F, De Paepe R, Remy R, Ambard-Bretteville F (1988) Regeneration of cytoplasmic male sterile protoclones of Nicotiana sylvestris with mitochondrial variations. Curr Genet 13:261–266
Liberman EA, Topaly VP, Tsofina LM, Jasaitis AA, Skulachev VP (1969) Mechanism of coupling of oxidative phosphorylation and the membrane potential of mitochondria. Nature 222:1076–1078
Lightowlers RN (2011) Mitochondrial transformation: time for concerted action. EMBO Rep 12:480–481
Lyrawati D, Trounson A, Cram D (2011) Expression of GFP in the mitochondrial compartment using DQAsome-mediated delivery of an artificial mini-mitochondrial genome. Pharm Res 28:2848–2862
Magalhaes PJ, Andreu AL, Schon EA (1998) Evidence for the presence of 5S rRNA in mammalian mitochondria. Mol Biol Cell 9:2375–2382
Mahata B, Mukherjee S, Mishra S, Bandyopadhyay A, Adhya S (2006) Functional delivery of a cytosolic tRNA into mutant mitochondria of human cells. Science 314:471–474
Mahato B, Jash S, Adhya S (2011) RNA-mediated restoration of mitochondrial function in cells harboring a Kearns Sayre Syndrome mutation. Mitochondrion 11:564–574
Malcuit I, Sorokin A (2010) Method for the transformation of plant cell mitochondria. Patent, Publication number WO/2010/061187, Application number PCT/GB2009/002755, Country GB
Manfredi G, Fu J, Ojaimi J, Sadlock JE, Kwong JQ, Guy J, Schon EA (2002) Rescue of a deficiency in ATP synthesis by transfer of MTATP6, a mitochondrial DNA-encoded gene, to the nucleus. Nat Genet 30:394–399
Marc P, Margeot A, Devaux F, Blugeon C, Corral-Debrinski M, Jacq C (2002) Genome-wide analysis of mRNAs targeted to yeast mitochondria. EMBO Rep 3:159–164
Maréchal-Drouard L, Guillemaut P, Cosset A, Arbogast M, Weber F, Weil JH, Dietrich A (1990) Transfer RNAs of potato (Solanum tuberosum) mitochondria have different genetic origins. Nucleic Acids Res 18:3689–3696
Matagne RF, Michel-Wolwertz MR, Munaut C, Duyckaerts C, Sluse F (1989) Induction and characterization of mitochondrial DNA mutants in Chlamydomonas reinhardtii. J Cell Biol 108:1221–1226
Matsuda D, Dreher TW (2004) The tRNA-like structure of Turnip yellow mosaic virus RNA is a 3’-translational enhancer. Virology 321:36–46
McDonald AE, Vanlerberghe GC, Staples JF (2009) Alternative oxidase in animals: unique characteristics and taxonomic distribution. J Exp Biol 212:2627–2634
Michaud M, Marechal-Drouard L, Duchene AM (2010) RNA trafficking in plant cells: targeting of cytosolic mRNAs to the mitochondrial surface. Plant Mol Biol 73:697–704
Mileshina D, Koulintchenko M, Konstantinov Y, Dietrich A (2011) Transfection of plant mitochondria and in organello gene integration. Nucleic Acids Res 39:e115
Miller-Messmer M, Kuhn K, Bichara M, Le Ret M, Imbault P, Gualberto JM (2012) RecA-dependent DNA repair results in increased heteroplasmy of the Arabidopsis mitochondrial genome. Plant Physiol 159:211–226
Minczuk M, Papworth MA, Kolasinska P, Murphy MP, Klug A (2006) Sequence-specific modification of mitochondrial DNA using a chimeric zinc finger methylase. Proc Natl Acad Sci U S A 103:19689–19694
Minczuk M, Papworth MA, Miller JC, Murphy MP, Klug A (2008) Development of a single-chain, quasi-dimeric zinc-finger nuclease for the selective degradation of mutated human mitochondrial DNA. Nucleic Acids Res 36:3926–3938
Minczuk M, Kolasinska-Zwierz P, Murphy MP, Papworth MA (2010) Construction and testing of engineered zinc-finger proteins for sequence-specific modification of mtDNA. Nat Protoc 5:342–356
Mireau H, Arnal N, Fox TD (2003) Expression of Barstar as a selectable marker in yeast mitochondria. Mol Genet Genomics 270:1–8
Mitchell P (1961) Coupling of phosphorylation to electron and hydrogen transfer by a chemi-osmotic type of mechanism. Nature 191:144–148
Moller SG, Xu XM (2009) Mitochondrial transformation. Patent, Publication number WO/2009/150441, Application number PCT/GB2009/001510, Country NO
Mossmann D, Meisinger C, Vogtle FN (2012) Processing of mitochondrial presequences. Biochim Biophys Acta 1819:1098–1106
Mukherjee S, Basu S, Home P, Dhar G, Adhya S (2007) Necessary and sufficient factors for the import of transfer RNA into the kinetoplast mitochondrion. EMBO Rep 8:589–595
Mukherjee S, Mahata B, Mahato B, Adhya S (2008) Targeted mRNA degradation by complex-mediated delivery of antisense RNAs to intracellular human mitochondria. Hum Mol Genet 17:1292–1298
Muratovska A, Lightowlers RN, Taylor RW, Turnbull DM, Smith RA, Wilce JA, Martin SW, Murphy MP (2001) 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 Res 29:1852–1863
Nagley P, Farrell LB, Gearing DP, Nero D, Meltzer S, Devenish RJ (1988) Assembly of functional proton-translocating ATPase complex in yeast mitochondria with cytoplasmically synthesized subunit 8, a polypeptide normally encoded within the organelle. Proc Natl Acad Sci U S A 85:2091–2095
Newman SM, Boynton JE, Gillham NW, Randolph-Anderson BL, Johnson AM, Harris EH (1990) Transformation of chloroplast ribosomal RNA genes in Chlamydomonas: molecular and genetic characterization of integration events. Genetics 126:875–888
Oca-Cossio J, Kenyon L, Hao H, Moraes CT (2003) Limitations of allotopic expression of mitochondrial genes in mammalian cells. Genetics 165:707–720
Ojaimi J, Pan J, Santra S, Snell WJ, Schon EA (2002) An algal nucleus-encoded subunit of mitochondrial ATP synthase rescues a defect in the analogous human mitochondrial-encoded subunit. Mol Biol Cell 13:3836–3844
Ortega VM, Bohner JG, Chase CD (2000) The tobacco apocytochrome b gene predicts sensitivity to the respiratory inhibitors antimycin A and myxothiazol. Curr Genet 37:315–321
Papworth M, Kolasinska P, Minczuk M (2006) Designer zinc-finger proteins and their applications. Gene 366:27–38
Parent JS, Lepage E, Brisson N (2011) Divergent roles for the two PolI-like organelle DNA polymerases of Arabidopsis. Plant Physiol 156:254–262
Paris Z, Rubio MA, Lukes J, Alfonzo JD (2009) Mitochondrial tRNA import in Trypanosoma brucei is independent of thiolation and the Rieske protein. RNA 15:1398–1406
Perales-Clemente E, Fernandez-Silva P, Acin-Perez R, Perez-Martos A, Enriquez JA (2011) Allotopic expression of mitochondrial-encoded genes in mammals: achieved goal, undemonstrated mechanism or impossible task? Nucleic Acids Res 39:225–234
Pineau B, Mathieu C, Gérard-Hirne C, De Paepe R, Chétrit P (2005) Targeting the NAD7 subunit to mitochondria restores a functional complex I and a wild type phenotype in the Nicotiana sylvestris CMS II mutant lacking nad7. J Biol Chem 280:25994–26001
Pinkert CA, Trounce IA (2002) Production of transmitochondrial mice. Methods 26:348–357
Pinkert CA, Irwin MH, Johnson LW, Moffatt RJ (1997) Mitochondria transfer into mouse ova by microinjection. Transgenic Res 6:379–383
Pohjoismaki JL, Holmes JB, Wood SR, Yang MY, Yasukawa T, Reyes A, Bailey LJ, Cluett TJ, Goffart S, Willcox S, Rigby RE, Jackson AP, Spelbrink JN, Griffith JD, Crouch RJ, Jacobs HT, Holt IJ (2010) Mammalian mitochondrial DNA replication intermediates are essentially duplex but contain extensive tracts of RNA/DNA hybrid. J Mol Biol 397:1144–1155
Qi X, Sun L, Lewin AS, Hauswirth WW, Guy J (2007) The mutant human ND4 subunit of complex I induces optic neuropathy in the mouse. Invest Ophthalmol Vis Sci 48:1–10
Randolph-Anderson BL, Boynton JE, Gillham NW, Harris EH, Johnson AM, Dorthu MP, Matagne RF (1993) Further characterization of the respiratory deficient dum-1 mutation of Chlamydomonas reinhardtii and its use as a recipient for mitochondrial transformation. Mol Gen Genet 236:235–244
Remacle C, Colin M, Matagne RF (1998) Suppression of a+ 1 T mutation by a nearby substitution in the mitochondrial cox1 gene of Chlamydomonas reinhardtii: a new type of frameshift suppression in an organelle genome. Mol Gen Genet 259:294–298
Remacle C, Duby F, Cardol P, Matagne RF (2001) Mutations inactivating mitochondrial genes in Chlamydomonas reinhardtii. Biochem Soc Trans 29:442–446
Remacle C, Cardol P, Coosemans N, Gaisne M, Bonnefoy N (2006) High-efficiency biolistic transformation of Chlamydomonas mitochondria can be used to insert mutations in complex I genes. Proc Natl Acad Sci U S A 103:4771–4776
Rinehart J, Krett B, Rubio MA, Alfonzo JD, Soll D (2005) Saccharomyces cerevisiae imports the cytosolic pathway for Gln-tRNA synthesis into the mitochondrion. Genes Dev 19:583–592
Rubio MA, Rinehart JJ, Krett B, Duvezin-Caubet S, Reichert AS, Soll D, Alfonzo JD (2008) Mammalian mitochondria have the innate ability to import tRNAs by a mechanism distinct from protein import. Proc Natl Acad Sci U S A 105:9186–9191
Ruhanen H, Ushakov K, Yasukawa T (2011) Involvement of DNA ligase III and ribonuclease H1 in mitochondrial DNA replication in cultured human cells. Biochim Biophys Acta 1813:2000–2007
Salinas T, Duchene AM, Marechal-Drouard L (2008) Recent advances in tRNA mitochondrial import. Trends Biochem Sci 33:320–329
Sbicego S, Nabholz CE, Hauser R, Blum B, Schneider A (1998) In vivo import of unspliced tRNATyr containing synthetic introns of variable length into mitochondria of Leishmania tarentolae. Nucleic Acids Res 26:5251–5255
Schekman R (2010) Editorial expression of concern: a bifunctional tRNA import receptor from Leishmania mitochondria. Proc Natl Acad Sci U S A 107:9476
Schneider A (2011) Mitochondrial tRNA import and its consequences for mitochondrial translation. Annu Rev Biochem 80:1033–1053
Schneider A, Maréchal-Drouard L (2000) Mitochondrial tRNA import: are there distinct mechanisms? Trends Cell Biol 10:509–513
Seibel P, Trappe J, Villani G, Klopstock T, Papa S, Reichmann H (1995) Transfection of mitochondria: strategy towards a gene therapy of mitochondrial DNA diseases. Nucleic Acids Res 23:10–17
Seibel M, Bachmann C, Schmiedel J, Wilken N, Wilde F, Reichmann H, Isaya G, Seibel P, Pfeiler D (1999) Processing of artificial peptide-DNA-conjugates by the mitochondrial intermediate peptidase (MIP). Biol Chem 380:961–967
Seo BB, Nakamaru-Ogiso E, Flotte TR, Matsuno-Yagi A, Yagi T (2006) In vivo complementation of complex I by the yeast Ndi1 enzyme. Possible application for treatment of Parkinson disease. J Biol Chem 281:14250–14255
Shadel GS, Clayton DA (1997) Mitochondrial DNA maintenance in vertebrates. Annu Rev Biochem 66:409–435
Shedge V, Arrieta-Montiel M, Christensen AC, Mackenzie SA (2007) Plant mitochondrial recombination surveillance requires unusual RecA and MutS homologs. Plant Cell 19:1251–1264
Sieber F, Placido A, El Farouk-AmeqraneS, Duchene AM, Marechal-Drouard L (2011) A protein shuttle system to target RNA into mitochondria. Nucleic Acids Res 39:e96
Simpson AM, Suyama Y, Dewes H, Campbell DA, Simpson L (1989) Kinetoplastid mitochondria contain functional tRNAs which are encoded in nuclear DNA and also contain small minicircle and maxicircle transcripts of unknown function. Nucleic Acids Res 17:5427–5445
Small I, Maréchal-Drouard L, Masson J, Pelletier G, Cosset A, Weil JH, Dietrich A (1992) In vivo import of a normal or mutagenized heterologous transfer RNA into the mitochondria of transgenic plants: towards novel ways of influencing mitochondrial gene expression? EMBO J 11:1291–1296
Smirnov A, Tarassov I, Mager-Heckel AM, Letzelter M, Martin RP, Krasheninnikov IA, Entelis N (2008) Two distinct structural elements of 5S rRNA are needed for its import into human mitochondria. RNA 14:749–759
Srivastava S, Moraes CT (2001) Manipulating mitochondrial DNA heteroplasmy by a mitochondrially targeted restriction endonuclease. Hum Mol Genet 10:3093–3099
Staudinger M, Kempken F (2003) Electroporation of isolated higher-plant mitochondria: transcripts of an introduced cox2 gene, but not an atp6 gene, are edited in organello. Mol Genet Genomics 269:553–561
Staudinger M, Bolle N, Kempken F (2005) Mitochondrial electroporation and in organello RNA editing of chimeric atp6 transcripts. Mol Genet Genomics 273:130–136
Steele DF, Butler CA, Fox TD (1996) Expression of a recoded nuclear gene inserted into yeast mitochondrial DNA is limited by mRNA-specific translational activation. Proc Natl Acad Sci U S A 93:5253–5257
Suyama Y (1986) Two dimensional polyacrylamide gel electrophoresis analysis of Tetrahymena mitochondrial tRNA. Curr Genet 10:411–420
Suzuki Y (2012) Exploring transduction mechanisms of protein transduction domains (PTDs) in living cells utilizing single-quantum dot tracking (SQT) technology. Sensors (Basel) 12:549–572
Svab Z, Maliga P (1993) High-frequency plastid transformation in tobacco by selection for a chimeric aadA gene. Proc Natl Acad Sci U S A 90:913–917
Svab Z, Hajdukiewicz P, Maliga P (1990) Stable transformation of plastids in higher plants. Proc Natl Acad Sci U S A 87:8526–8530
Sylvestre J, Margeot A, Jacq C, Dujardin G, Corral-Debrinski M (2003) The role of the 3’ untranslated region in mRNA sorting to the vicinity of mitochondria is conserved from yeast to human cells. Mol Biol Cell 14:3848–3856
Tachibana M, Amato P, Sparman M, Woodward J, Sanchis DM, Ma H, Gutierrez NM, Tippner-Hedges R, Kang E, Lee HS, Ramsey C, Masterson K, Battaglia D, Lee D, Wu D, Jensen J, Patton P, Gokhale S, Stouffer R, Mitalipov S (2012) Towards germline gene therapy of inherited mitochondrial diseases. Nature. doi:10.1038/nature11647
Tanaka M, Borgeld HJ, Zhang J, Muramatsu S, Gong JS, Yoneda M, Maruyama W, Naoi M, Ibi T, Sahashi K, Shamoto M, Fuku N, Kurata M, Yamada Y, Nishizawa K, Akao Y, Ohishi N, Miyabayashi S, Umemoto H, Muramatsu T, Furukawa K, Kikuchi A, Nakano I, Ozawa K, Yagi K (2002) Gene therapy for mitochondrial disease by delivering restriction endonuclease SmaI into mitochondria. J Biomed Sci 9:534–541
Tarassov IA, Entelis NS (1992) Mitochondrially-imported cytoplasmic tRNA(Lys)(CUU) of Saccharomyces cerevisiae: in vivo and in vitro targetting systems. Nucleic Acids Res 20:1277–1281
Thomas RR, Khan SM, Portell FR, Smigrodzki RM, Bennett JP Jr (2011) Recombinant human mitochondrial transcription factor A stimulates mitochondrial biogenesis and ATP synthesis, improves motor function after MPTP, reduces oxidative stress and increases survival after endotoxin. Mitochondrion 11:108–118
Vahrenholz C, Riemen G, Pratje E, Dujon B, Michaelis G (1993) Mitochondrial DNA of Chlamydomonas reinhardtii: the structure of the ends of the linear 15.8-kb genome suggests mechanisms for DNA replication. Curr Genet 24:241–247
Val R, Wyszko E, Valentin C, Szymanski M, Cosset A, Alioua M, Dreher TW, Barciszewski J, Dietrich A (2011) Organelle trafficking of chimeric ribozymes and genetic manipulation of mitochondria. Nucleic Acids Res 39:9262–9274
Van Aken O, Giraud E, Clifton R, Whelan J (2009) Alternative oxidase: a target and regulator of stress responses. Physiol Plant 137:354–361
Vestweber D, Schatz G (1989) DNA-protein conjugates can enter mitochondria via the protein import pathway. Nature 338:170–172
von Allmen JM, Rottmann WH, Gengenbach BG, Harvey AJ, Lonsdale DM (1991) Transfer of methomyl and HmT-toxin sensitivity from T-cytoplasm maize to tobacco. Mol Gen Genet 229:405–412
Wagle MA, Martinville LE, D’Souza GG (2011) The utility of an isolated mitochondrial fraction in the preparation of liposomes for the specific delivery of bioactives to mitochondria in live mammalian cells. Pharm Res 28:2790–2796
Wang Z, Zou Y, Li X, Zhang Q, Chen L, Wu H, Su D, Chen Y, Guo J, Luo D, Long Y, Zhong Y, Liu YG (2006) Cytoplasmic male sterility of rice with boro II cytoplasm is caused by a cytotoxic peptide and is restored by two related PPR motif genes via distinct modes of mRNA silencing. Plant Cell 18:676–687
Wang G, Chen HW, Oktay Y, Zhang J, Allen EL, Smith GM, Fan KC, Hong JS, French SW, McCaffery JM, Lightowlers RN, Morse HC, 3rd, Koehler CM, Teitell MA (2010) PNPASE regulates RNA import into mitochondria. Cell 142:456–467
Wang G, Shimada E, Koehler CM, Teitell MA (2012a) PNPASE and RNA trafficking into mitochondria. Biochim Biophys Acta 1819:998–1007
Wang G, Shimada E, Zhang J, Hong JS, Smith GM, Teitell MA, Koehler CM (2012b) Correcting human mitochondrial mutations with targeted RNA import. Proc Natl Acad Sci U S A 109:4840–4845
Wanrooij S, Miralles Fuste J, Stewart JB, Wanrooij PH, Samuelsson T, Larsson NG, Gustafsson CM, Falkenberg M (2012) In vivo mutagenesis reveals that OriL is essential for mitochondrial DNA replication. EMBO Rep. doi:10.1038/embor.2012.1161
Waters VL (1999) Conjugative transfer in the dissemination of beta-lactam and aminoglycoside resistance. Front Biosci 4:D433–D456
Weber-Lotfi F, Ibrahim N, Boesch P, Cosset A, Konstantinov Y, Lightowlers RN, Dietrich A (2009) Developing a genetic approach to investigate the mechanism of mitochondrial competence for DNA import. Biochim Biophys Acta 1787:320–327
Weissig V, Lasch J, Erdos G, Meyer HW, Rowe TC, Hughes J (1998) DQAsomes: a novel potential drug and gene delivery system made from Dequalinium. Pharm Res 15:334–337
Weissig V, Lizano C, Torchilin VP (2000) Selective DNA release from DQAsome/DNA complexes at mitochondria-like membranes. Drug Deliv 7:1–5
Weissig V, D’Souza GG, Torchilin VP (2001) DQAsome/DNA complexes release DNA upon contact with isolated mouse liver mitochondria. J Control Release 75:401–408
Weissig V, Boddapati SV, Cheng SM, D’Souza GG (2006) Liposomes and liposome-like vesicles for drug and DNA delivery to mitochondria. J Liposome Res 16:249–264
Wintz H, Chen HC, Sutton CA, Conley CA, Cobb A, Ruth D, Hanson MR (1995) Expression of the CMS-associated urfS sequence in transgenic petunia and tobacco. Plant Mol Biol 28:83–92
Yagi T, Seo BB, Nakamaru-Ogiso E, Marella M, Barber-Singh J, Yamashita T, Kao MC, Matsuno-Yagi A (2006a) Can a single subunit yeast NADH dehydrogenase (Ndi1) remedy diseases caused by respiratory complex I defects? Rejuvenation Res 9:191–197
Yagi T, Seo BB, Nakvamaru-Ogiso E, Marella M, Barber-Singh J, Yamashita T, Matsuno-Yagi A (2006b) Possibility of transkingdom gene therapy for complex I diseases. Biochim Biophys Acta 1757:708–714
Yamada Y, Harashima H (2012a) Delivery of bioactive molecules to the mitochondrial genome using a membrane-fusing, liposome-based carrier, DF-MITO-Porter. Biomaterials 33:1589–1595
Yamada Y, Harashima H (2012b) Enhancement in selective mitochondrial association by direct modification of a mitochondrial targeting signal peptide on a liposomal based nanocarrier. Mitochondrion. doi:10.1016/j.mito.2012.1009.1001
Yamada Y, Akita H, Kamiya H, Kogure K, Yamamoto T, Shinohara Y, Yamashita K, Kobayashi H, Kikuchi H, Harashima H (2008) MITO-Porter: a liposome-based carrier system for delivery of macromolecules into mitochondria via membrane fusion. Biochim Biophys Acta 1778:423–432
Yamada Y, Furukawa R, Yasuzaki Y, Harashima H (2011) Dual function MITO-Porter, a nano carrier integrating both efficient cytoplasmic delivery and mitochondrial macromolecule delivery. Mol Ther 19:1449–1456
Yamamoto MP, Shinada H, Onodera Y, Komaki C, Mikami T, Kubo T (2008) A male sterility-associated mitochondrial protein in wild beets causes pollen disruption in transgenic plants. Plant J 54:1027–1036
Yamasaki T, Kurokawa S, Watanabe KI, Ikuta K, Ohama T (2005) Shared molecular characteristics of successfully transformed mitochondrial genomes in Chlamydomonas reinhardtii. Plant Mol Biol 58:515–527
Yang YW, Koob MD (2012) Transferring isolated mitochondria into tissue culture cells. Nucleic Acids Res. doi:10.1093/nar/gks1639
Yang MY, Bowmaker M, Reyes A, Vergani L, Angeli P, Gringeri E, Jacobs HT, Holt IJ (2002) Biased incorporation of ribonucleotides on the mitochondrial L-strand accounts for apparent strand-asymmetric DNA replication. Cell 111:495–505
Yang J, Liu X, Yang X, Zhang M (2010) Mitochondrially-targeted expression of a cytoplasmic male sterility-associated orf220 gene causes male sterility in Brassica juncea. BMC Plant Biol 10:231
Yasukawa T, Yang MY, Jacobs HT, Holt IJ (2005) A bidirectional origin of replication maps to the major noncoding region of human mitochondrial DNA. Mol Cell 18:651–662
Yasuzaki Y, Yamada Y, Harashima H (2010) Mitochondrial matrix delivery using MITO-Porter, a liposome-based carrier that specifies fusion with mitochondrial membranes. Biochem Biophys Res Commun 397:181–186
Yoon YG, Koob MD (2003) Efficient cloning and engineering of entire mitochondrial genomes in Escherichia coli and transfer into transcriptionally active mitochondria. Nucleic Acids Res 31:1407–1415
Yoon YG, Koob MD (2005) Transformation of isolated mammalian mitochondria by bacterial conjugation. Nucleic Acids Res 33:e139
Yoon YG, Koob MD (2008) Selection by drug resistance proteins located in the mitochondria of mammalian cells. Mitochondrion 8:345–351
Yoon YG, Koob MD (2011) Toward genetic transformation of mitochondria in mammalian cells using a recoded drug-resistant selection marker. J Genet Genomics 38:173–179
Yoon YG, Koob MD (2012) Nonreplicating intracellular bacterial vector for conjugative DNA transfer into mitochondria. Pharm Res 29:1040–1045
Yoon YG, Yang YW, Koob MD (2009) PCR-based cloning of the complete mouse mitochondrial genome and stable engineering in Escherichia coli. Biotechnol Lett 31:1671–1676
Yoshionari S, Koike T, Yokogawa T, Nishikawa K, Ueda T, Miura K, Watanabe K (1994) Existence of nuclear-encoded 5S-rRNA in bovine mitochondria. FEBS Lett 338:137–142
Yu H, Koilkonda RD, Chou TH, Porciatti V, Ozdemir SS, Chiodo V, Boye SL, Boye SE, Hauswirth WW, Lewin AS, Guy J (2012a) Gene delivery to mitochondria by targeting modified adenoassociated virus suppresses Leber’s hereditary optic neuropathy in a mouse model. Proc Natl Acad Sci U S A 109:E1238–E1247
Yu H, Ozdemir SS, Koilkonda RD, Chou TH, Porciatti V, Chiodo V, Boye SL, Hauswirth WW, Lewin AS, Guy J (2012b) Mutant NADH dehydrogenase subunit 4 gene delivery to mitochondria by targeting sequence-modified adeno-associated virus induces visual loss and optic atrophy in mice. Mol Vis 18:1668–1683
Zaegel V, Guermann B, Le Ret M, Andres C, Meyer D, Erhardt M, Canaday J, Gualberto JM, Imbault P (2006) The plant-specific ssDNA binding protein OSB1 is involved in the stoichiometric transmission of mitochondrial DNA in Arabidopsis. Plant Cell 18:3548–3563
Zhou J, Liu L, Chen J (2010) Mitochondrial DNA heteroplasmy in Candida glabrata after mitochondrial transformation. Eukaryot Cell 9:806–814
Acknowledgments
Collaborations with the laboratories of Y. Konstantinov (SIFIBR, Irkutsk, Russia), R.N. Lightowlers (University of Newcastle, UK), G.G.M. D’Souza (MCPHS, Boston, USA) and J. Barciszewski (IBCH, Poznan, Poland) are kindly acknowledged. Our projects are funded by the French Centre National de la Recherche Scientifique (CNRS, UPR2357), the Université de Strasbourg (UdS), the Agence Nationale de la Recherche (ANR-06-MRAR-037-02, ANR-09-BLAN-0240-01) and the Ministère de la Recherche et de l’Enseignement Supérieur (Investissements d’Avenir/Laboratoire d’Excellence MitoCross).
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2015 Springer Science+Business Media, LLC
About this chapter
Cite this chapter
Mileshina, D., Niazi, A., Weber-Lotfi, F., Gualberto, J., Dietrich, A. (2015). Mitochondrial Genetic Manipulation. In: Li, XQ., Donnelly, D., Jensen, T. (eds) Somatic Genome Manipulation. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-2389-2_13
Download citation
DOI: https://doi.org/10.1007/978-1-4939-2389-2_13
Published:
Publisher Name: Springer, New York, NY
Print ISBN: 978-1-4939-2388-5
Online ISBN: 978-1-4939-2389-2
eBook Packages: Biomedical and Life SciencesBiomedical and Life Sciences (R0)