<?xml version="1.0" encoding="UTF-8"?>
<!DOCTYPE article PUBLIC "-//NLM//DTD JATS (Z39.96) Journal Publishing DTD v1.3 20210610//EN" "JATS-journalpublishing1-3.dtd">
<article article-type="research-article" dtd-version="1.3" xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" xml:lang="ru"><front><journal-meta><journal-id journal-id-type="publisher-id">vestnik-bio-msu</journal-id><journal-title-group><journal-title xml:lang="ru">Вестник Московского университета. Серия 16. Биология</journal-title><trans-title-group xml:lang="en"><trans-title>Vestnik Moskovskogo universiteta. Seriya 16. Biologiya</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">0137-0952</issn><publisher><publisher-name>Lomonosov Moscow State University,  School of Biology</publisher-name></publisher></journal-meta><article-meta><article-id custom-type="elpub" pub-id-type="custom">vestnik-bio-msu-271</article-id><article-categories><subj-group subj-group-type="heading"><subject>Research Article</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="ru"><subject>Молекулярная биология</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>Molecular biology</subject></subj-group></article-categories><title-group><article-title>ИМПОРТ РНК В МИТОХОНДРИИ И ЕГО ПРИМЕНЕНИЕ В ГЕННОЙ ТЕРАПИИ</article-title><trans-title-group xml:lang="en"><trans-title>RNA IMPORT INTO MITOCHONDRIA AND ITS USE IN THE GENE THERAPY</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Лакунина</surname><given-names>Валентина Александровна</given-names></name><name name-style="western" xml:lang="en"><surname>Lakunina</surname><given-names>V. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>аспирант кафедры молекулярной биологии</p></bio><email xlink:type="simple">carrie8@yandex.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Балева</surname><given-names>Мария Вячеславовна</given-names></name><name name-style="western" xml:lang="en"><surname>Baleva</surname><given-names>M. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>аспирант кафедры молекулярной биологии</p></bio><email xlink:type="simple">mary-bw@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Левицкий</surname><given-names>Сергей Алексеевич</given-names></name><name name-style="western" xml:lang="en"><surname>Levitskii</surname><given-names>S. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>канд. биол. наук, ст. науч. сотр. кафедры молекулярной биологии</p></bio><email xlink:type="simple">krolick@yandex.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Чичерин</surname><given-names>Иван Владимирович</given-names></name><name name-style="western" xml:lang="en"><surname>Chicherin</surname><given-names>I. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>аспирант кафедры молекулярной биологии</p></bio><email xlink:type="simple">i.v.chicherin@gmail.com</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Крашенинников</surname><given-names>Игорь Александрович</given-names></name><name name-style="western" xml:lang="en"><surname>Krasheninnikov</surname><given-names>I. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>канд биол. наук, проф. кафедры молекулярной биологии</p></bio><email xlink:type="simple">igor@protrin.bio.msu.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Самойлова</surname><given-names>Елена Олеговна</given-names></name><name name-style="western" xml:lang="en"><surname>Samoylova</surname><given-names>E. O.</given-names></name></name-alternatives><bio xml:lang="ru"><p>мл. науч. сотр. кафедры молекулярной биологии</p></bio><email xlink:type="simple">elen.samoylova@yandex.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Каменский</surname><given-names>Пётр Андреевич</given-names></name><name name-style="western" xml:lang="en"><surname>Kamenski</surname><given-names>P. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>канд. биол. наук, вед. науч. сотр. кафедры молекулярной биологии</p></bio><email xlink:type="simple">piotr.kamenski@gmail.com</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff xml:lang="ru" id="aff-1"><institution>биологический факультет МГУ</institution><country>Russian Federation</country></aff><pub-date pub-type="collection"><year>2015</year></pub-date><pub-date pub-type="epub"><day>17</day><month>08</month><year>2015</year></pub-date><volume>0</volume><issue>3</issue><fpage>32</fpage><lpage>36</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Лакунина В.А., Балева М.В., Левицкий С.А., Чичерин И.В., Крашенинников И.А., Самойлова Е.О., Каменский П.А., 2015</copyright-statement><copyright-year>2015</copyright-year><copyright-holder xml:lang="ru">Лакунина В.А., Балева М.В., Левицкий С.А., Чичерин И.В., Крашенинников И.А., Самойлова Е.О., Каменский П.А.</copyright-holder><copyright-holder xml:lang="en">Lakunina V.A., Baleva M.V., Levitskii S.A., Chicherin I.V., Krasheninnikov I.A., Samoylova E.O., Kamenski P.A.</copyright-holder><license xml:lang="ru" license-type="creative-commons-attribution" xlink:href="https://creativecommons.org/licenses/by/4.0/" xlink:type="simple"><license-p>Данная работа распространяется под лицензией Creative Commons Attribution 4.0.</license-p></license><license xml:lang="en" license-type="creative-commons-attribution" xlink:href="https://creativecommons.org/licenses/by/4.0/" xlink:type="simple"><license-p>This work is licensed under a Creative Commons Attribution 4.0 License.</license-p></license></permissions><self-uri xlink:href="https://vestnik-bio-msu.elpub.ru/jour/article/view/271">https://vestnik-bio-msu.elpub.ru/jour/article/view/271</self-uri><abstract><p>К биологическим макромолекулам, импортирующимся в митохондрии эукариотических клеток из цитозоля, относятся белки и рибонуклеиновые кислоты. Импорт белков в митохондрии — универсальный процесс, и его механизмы описаны в достаточной степени подробно. Импорт РНК в митохондрии, напротив, показан только для некоторых групп организмов, и в каждом случае механизмы данного процесса обладают большим количеством индивидуальных особенностей. В данном обзоре рассматриваются современные данные о процессе импорта РНК в митохондрии клеток человека и обсуждается возможность использования данного процесса в генной терапии митохондриальных болезней.</p></abstract><trans-abstract xml:lang="en"><p>Among biological macromolecules imported into the eukaryotic cells mitochondria, there are proteins and ribonucleic acids. Protein import into mitochondria is a universal process, and its mechanisms are currently well described. RNA import into mitochondria, on the contrary, is known only for several eukaryotic species, and the mechanisms of this process are largely species-specific. In this review, we summarize existing data about RNA import into mitochondria of human cells and discuss the possibilities of its implication for the gene therapy of mitochondrial diseases.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>митохондрии</kwd><kwd>РНК</kwd><kwd>импорт РНК в митохондрии</kwd><kwd>генная терапия</kwd><kwd>обзор</kwd></kwd-group><kwd-group xml:lang="en"><kwd>mitochondria</kwd><kwd>RNA</kwd><kwd>RNA import into mitochondria</kwd><kwd>gene therapy</kwd><kwd>review</kwd></kwd-group><funding-group><funding-statement xml:lang="ru">Министерство образования и науки РФ</funding-statement></funding-group></article-meta></front><back><ref-list><title>References</title><ref id="cit1"><label>1</label><citation-alternatives><mixed-citation xml:lang="ru">Schneider A. Mitochondrial tRNA import and its consequences for mitochondrial translation // Annu. Rev. Biochem. 2011. Vol. 80. P. 1033—1053.</mixed-citation><mixed-citation xml:lang="en">Schneider A. Mitochondrial tRNA import and its consequences for mitochondrial translation // Annu. Rev. Biochem. 2011. Vol. 80. P. 1033—1053.</mixed-citation></citation-alternatives></ref><ref id="cit2"><label>2</label><citation-alternatives><mixed-citation xml:lang="ru">Chang D.D., Clayton D.A. A mammalian mitochondrial RNA processing activity contains nucleus-encoded RNA // Science. 1987. Vol. 235. N 4793. P. 1178—1184.</mixed-citation><mixed-citation xml:lang="en">Chang D.D., Clayton D.A. A mammalian mitochondrial RNA processing activity contains nucleus-encoded RNA // Science. 1987. Vol. 235. N 4793. P. 1178—1184.</mixed-citation></citation-alternatives></ref><ref id="cit3"><label>3</label><citation-alternatives><mixed-citation xml:lang="ru">Li K., Smagula C.S., Parsons W.J., Richardson J.A., Gonzalez M., Hagler H.K., Williams R.S. Subcellular partitioning of MRP RNA assessed by ultrastructural and biochemical analysis // J. Cell Biol. 1994. Vol. 124. N 6. P. 871—882.</mixed-citation><mixed-citation xml:lang="en">Li K., Smagula C.S., Parsons W.J., Richardson J.A., Gonzalez M., Hagler H.K., Williams R.S. Subcellular partitioning of MRP RNA assessed by ultrastructural and biochemical analysis // J. Cell Biol. 1994. Vol. 124. N 6. P. 871—882.</mixed-citation></citation-alternatives></ref><ref id="cit4"><label>4</label><citation-alternatives><mixed-citation xml:lang="ru">Doersen C.J., Guerrier-Takada C., Altman S., Attardi G. Characterization of an RNase P activity from HeLa cell mitochondria. Comparison with the cytosol RNase P activity // J. Biol. Chem. 1985. Vol. 260. N 10. P. 5942—5949.</mixed-citation><mixed-citation xml:lang="en">Doersen C.J., Guerrier-Takada C., Altman S., Attardi G. Characterization of an RNase P activity from HeLa cell mitochondria. Comparison with the cytosol RNase P activity // J. Biol. Chem. 1985. Vol. 260. N 10. P. 5942—5949.</mixed-citation></citation-alternatives></ref><ref id="cit5"><label>5</label><citation-alternatives><mixed-citation xml:lang="ru">Wang G., Chen H.W., Oktay Y., Zhang J., Allen E.L., Smith G.M., Fan K.C., Hong J.S., French S.W., McCaffery J.M., Lightowlers R.N., Morse H.C. III, Koehler C.M., Teitell M.A. PNPASE regulates RNA import into mitochondria // Cell. 2010. Vol. 142. N 3. P. 456—467.</mixed-citation><mixed-citation xml:lang="en">Wang G., Chen H.W., Oktay Y., Zhang J., Allen E.L., Smith G.M., Fan K.C., Hong J.S., French S.W., McCaffery J.M., Lightowlers R.N., Morse H.C. III, Koehler C.M., Teitell M.A. PNPASE regulates RNA import into mitochondria // Cell. 2010. Vol. 142. N 3. P. 456—467.</mixed-citation></citation-alternatives></ref><ref id="cit6"><label>6</label><citation-alternatives><mixed-citation xml:lang="ru">Wang G., Shimada E., Koehler C.M., Teitell M.A. PNPASE and RNA trafficking into mitochondria // Biochim. Biophys. Acta. 2012. Vol. 1819. N 9—10. P. 998—1007.</mixed-citation><mixed-citation xml:lang="en">Wang G., Shimada E., Koehler C.M., Teitell M.A. PNPASE and RNA trafficking into mitochondria // Biochim. Biophys. Acta. 2012. Vol. 1819. N 9—10. P. 998—1007.</mixed-citation></citation-alternatives></ref><ref id="cit7"><label>7</label><citation-alternatives><mixed-citation xml:lang="ru">Rainey R.N., Glavin J.D., Chen H.W., French S.W., Teitell M.A., Koehler C.M. A new function in translocation for the mitochondrial i-AAA protease Yme1: import of polynucleotide phosphorylase into the intermembrane space // Mol. Cell Biol. 2006. Vol. 26. N 22. P. 8488—8497.</mixed-citation><mixed-citation xml:lang="en">Rainey R.N., Glavin J.D., Chen H.W., French S.W., Teitell M.A., Koehler C.M. A new function in translocation for the mitochondrial i-AAA protease Yme1: import of polynucleotide phosphorylase into the intermembrane space // Mol. Cell Biol. 2006. Vol. 26. N 22. P. 8488—8497.</mixed-citation></citation-alternatives></ref><ref id="cit8"><label>8</label><citation-alternatives><mixed-citation xml:lang="ru">Wang G., Shimada E., Zhang J., Hong J.S., Smith G.M., Teitell M.A., Koehler C.M. Correcting human mitochondrial mutations with targeted RNA import // Proc. Natl. Acad. Sci. USA. 2012. Vol. 109. N 13. P. 4840—4845.</mixed-citation><mixed-citation xml:lang="en">Wang G., Shimada E., Zhang J., Hong J.S., Smith G.M., Teitell M.A., Koehler C.M. Correcting human mitochondrial mutations with targeted RNA import // Proc. Natl. Acad. Sci. USA. 2012. Vol. 109. N 13. P. 4840—4845.</mixed-citation></citation-alternatives></ref><ref id="cit9"><label>9</label><citation-alternatives><mixed-citation xml:lang="ru">Yoshionari S., Koike T., Yokogawa T., Nishikawa K., Ueda T., Miura K., Watanabe K. Existence of nuclearencoded 5S-rRNA in bovine mitochondria // FEBS Lett. 1994. Vol. 338. N 2. P. 137—142.</mixed-citation><mixed-citation xml:lang="en">Yoshionari S., Koike T., Yokogawa T., Nishikawa K., Ueda T., Miura K., Watanabe K. Existence of nuclearencoded 5S-rRNA in bovine mitochondria // FEBS Lett. 1994. Vol. 338. N 2. P. 137—142.</mixed-citation></citation-alternatives></ref><ref id="cit10"><label>10</label><citation-alternatives><mixed-citation xml:lang="ru">Magalhaes P.J., Andreu A.L., Schon E.A. Evidence for the presence of 5S rRNA in mammalian mitochondria // Mol. Biol. Cell. 1998. Vol. 9. N 9. P. 2375—2382.</mixed-citation><mixed-citation xml:lang="en">Magalhaes P.J., Andreu A.L., Schon E.A. Evidence for the presence of 5S rRNA in mammalian mitochondria // Mol. Biol. Cell. 1998. Vol. 9. N 9. P. 2375—2382.</mixed-citation></citation-alternatives></ref><ref id="cit11"><label>11</label><citation-alternatives><mixed-citation xml:lang="ru">Smirnov A.V., Entelis N.S., Krasheninnikov I.A., Martin R., Tarassov I.A. Specific features of 5S rRNA structure — its interactions with macromolecules and possible functions // Biochemistry (Mosc.). 2008. Vol. 73. N 13. P. 1418—1437.</mixed-citation><mixed-citation xml:lang="en">Smirnov A.V., Entelis N.S., Krasheninnikov I.A., Martin R., Tarassov I.A. Specific features of 5S rRNA structure — its interactions with macromolecules and possible functions // Biochemistry (Mosc.). 2008. Vol. 73. N 13. P. 1418—1437.</mixed-citation></citation-alternatives></ref><ref id="cit12"><label>12</label><citation-alternatives><mixed-citation xml:lang="ru">Smirnov A., Tarassov I., Mager-Heckel A.M., Letzelter M., Martin R.P., Krasheninnikov I.A., Entelis N. Two distinct structural elements of 5S rRNA are needed for its import into human mitochondria // RNA. 2008. Vol. 14. N 4. P. 749—759.</mixed-citation><mixed-citation xml:lang="en">Smirnov A., Tarassov I., Mager-Heckel A.M., Letzelter M., Martin R.P., Krasheninnikov I.A., Entelis N. Two distinct structural elements of 5S rRNA are needed for its import into human mitochondria // RNA. 2008. Vol. 14. N 4. P. 749—759.</mixed-citation></citation-alternatives></ref><ref id="cit13"><label>13</label><citation-alternatives><mixed-citation xml:lang="ru">Entelis N.S., Kolesnikova O.A., Dogan S., Martin R.P., Tarassov I.A. 5S rRNA and tRNA import into human mitochondria. Comparison of in vitro requirements // J. Biol. Chem. 2001. Vol. 276. N 49. P. 45642—45653.</mixed-citation><mixed-citation xml:lang="en">Entelis N.S., Kolesnikova O.A., Dogan S., Martin R.P., Tarassov I.A. 5S rRNA and tRNA import into human mitochondria. Comparison of in vitro requirements // J. Biol. Chem. 2001. Vol. 276. N 49. P. 45642—45653.</mixed-citation></citation-alternatives></ref><ref id="cit14"><label>14</label><citation-alternatives><mixed-citation xml:lang="ru">Smirnov A., Comte C., Mager-Heckel A.M., Addis V., Krasheninnikov I.A., Martin R.P., Entelis N., Tarassov I. Mitochondrial enzyme rhodanese is essential for 5S ribosomal RNA import into human mitochondria // J. Biol. Chem. 2010. Vol. 285. N 40. P. 30792—30803.</mixed-citation><mixed-citation xml:lang="en">Smirnov A., Comte C., Mager-Heckel A.M., Addis V., Krasheninnikov I.A., Martin R.P., Entelis N., Tarassov I. Mitochondrial enzyme rhodanese is essential for 5S ribosomal RNA import into human mitochondria // J. Biol. Chem. 2010. Vol. 285. N 40. P. 30792—30803.</mixed-citation></citation-alternatives></ref><ref id="cit15"><label>15</label><citation-alternatives><mixed-citation xml:lang="ru">Smirnov A., Entelis N., Martin R.P., Tarassov I. Biological significance of 5S rRNA import into human mitochondria: role of ribosomal protein MRP-L18 // Genes Dev. 2011. Vol. 25. N 12. P. 1289—1305.</mixed-citation><mixed-citation xml:lang="en">Smirnov A., Entelis N., Martin R.P., Tarassov I. Biological significance of 5S rRNA import into human mitochondria: role of ribosomal protein MRP-L18 // Genes Dev. 2011. Vol. 25. N 12. P. 1289—1305.</mixed-citation></citation-alternatives></ref><ref id="cit16"><label>16</label><citation-alternatives><mixed-citation xml:lang="ru">Entelis N., Brandina I., Kamenski P., Krasheninnikov I.A., Martin R.P., Tarassov I. A glycolytic enzyme, enolase, is recruited as a cofactor of tRNA targeting toward mitochondria in Saccharomyces cerevisiae // Genes Dev. 2006. Vol. 20. N 12. P. 1609—1620.</mixed-citation><mixed-citation xml:lang="en">Entelis N., Brandina I., Kamenski P., Krasheninnikov I.A., Martin R.P., Tarassov I. A glycolytic enzyme, enolase, is recruited as a cofactor of tRNA targeting toward mitochondria in Saccharomyces cerevisiae // Genes Dev. 2006. Vol. 20. N 12. P. 1609—1620.</mixed-citation></citation-alternatives></ref><ref id="cit17"><label>17</label><citation-alternatives><mixed-citation xml:lang="ru">Kamenski P., Smirnova E., Kolesnikova O., Krasheninnikov I.A., Martin R.P., Entelis N., Tarassov I. tRNA mitochondrial import in yeast: Mapping of the import determinants in the carrier protein, the precursor of mitochondrial lysyl-tRNA synthetase // Mitochondrion. 2010. Vol. 10. N 3. P. 284—293.</mixed-citation><mixed-citation xml:lang="en">Kamenski P., Smirnova E., Kolesnikova O., Krasheninnikov I.A., Martin R.P., Entelis N., Tarassov I. tRNA mitochondrial import in yeast: Mapping of the import determinants in the carrier protein, the precursor of mitochondrial lysyl-tRNA synthetase // Mitochondrion. 2010. Vol. 10. N 3. P. 284—293.</mixed-citation></citation-alternatives></ref><ref id="cit18"><label>18</label><citation-alternatives><mixed-citation xml:lang="ru">Brown A., Amunts A., Bai X. C., Sugimoto Y., Edwards P.C., Murshudov G., Scheres S.H., Ramakrishnan V. Structure of the large ribosomal subunit from human mitochondria // Science. 2014. Vol. 346. N 6210. P. 718—722.</mixed-citation><mixed-citation xml:lang="en">Brown A., Amunts A., Bai X. C., Sugimoto Y., Edwards P.C., Murshudov G., Scheres S.H., Ramakrishnan V. Structure of the large ribosomal subunit from human mitochondria // Science. 2014. Vol. 346. N 6210. P. 718—722.</mixed-citation></citation-alternatives></ref><ref id="cit19"><label>19</label><citation-alternatives><mixed-citation xml:lang="ru">Greber B.J., Boehringer D., Leibundgut M., Bieri P., Leitner A., Schmitz N., Aebersold R., Ban N. The complete structure of the large subunit of the mammalian mitochondrial ribosome // Nature. 2014. Vol. 515. N 7526. P. 283—286.</mixed-citation><mixed-citation xml:lang="en">Greber B.J., Boehringer D., Leibundgut M., Bieri P., Leitner A., Schmitz N., Aebersold R., Ban N. The complete structure of the large subunit of the mammalian mitochondrial ribosome // Nature. 2014. Vol. 515. N 7526. P. 283—286.</mixed-citation></citation-alternatives></ref><ref id="cit20"><label>20</label><citation-alternatives><mixed-citation xml:lang="ru">Gowher A., Smirnov A., Tarassov I., Entelis N. Induced tRNA import into human mitochondria: implication of a host aminoacyl-tRNA-synthetase // PLoS One. 2013. Vol. 8. N 6. P. e66228.</mixed-citation><mixed-citation xml:lang="en">Gowher A., Smirnov A., Tarassov I., Entelis N. Induced tRNA import into human mitochondria: implication of a host aminoacyl-tRNA-synthetase // PLoS One. 2013. Vol. 8. N 6. P. e66228.</mixed-citation></citation-alternatives></ref><ref id="cit21"><label>21</label><citation-alternatives><mixed-citation xml:lang="ru">Kolesnikova O.A., Entelis N.S., Jacquin-Becker C., Goltzene F., Chrzanowska-Lightowlers Z.M., Lightowlers R.N., Martin R.P., Tarassov I. 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. 2004. Vol. 13. N 20. P. 2519—2534.</mixed-citation><mixed-citation xml:lang="en">Kolesnikova O.A., Entelis N.S., Jacquin-Becker C., Goltzene F., Chrzanowska-Lightowlers Z.M., Lightowlers R.N., Martin R.P., Tarassov I. 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. 2004. Vol. 13. N 20. P. 2519—2534.</mixed-citation></citation-alternatives></ref><ref id="cit22"><label>22</label><citation-alternatives><mixed-citation xml:lang="ru">Patrushev M.V., Kamenski P.A., Mazunin I.O. Mutations in mitochondrial DNA and approaches for their correction // Biochemistry (Mosk.). 2014. Vol. 79. N 11. P. 1151—1160.</mixed-citation><mixed-citation xml:lang="en">Patrushev M.V., Kamenski P.A., Mazunin I.O. Mutations in mitochondrial DNA and approaches for their correction // Biochemistry (Mosk.). 2014. Vol. 79. N 11. P. 1151—1160.</mixed-citation></citation-alternatives></ref><ref id="cit23"><label>23</label><citation-alternatives><mixed-citation xml:lang="ru">Kolesnikova O.A., Entelis N.S., Mireau H., Fox T.D., Martin R.P., Tarassov I.A. Suppression of mutations in mitochondrial DNA by tRNAs imported from the cytoplasm // Science. 2000. Vol. 289. N 5486. P. 1931—1933.</mixed-citation><mixed-citation xml:lang="en">Kolesnikova O.A., Entelis N.S., Mireau H., Fox T.D., Martin R.P., Tarassov I.A. Suppression of mutations in mitochondrial DNA by tRNAs imported from the cytoplasm // Science. 2000. Vol. 289. N 5486. P. 1931—1933.</mixed-citation></citation-alternatives></ref><ref id="cit24"><label>24</label><citation-alternatives><mixed-citation xml:lang="ru">Karicheva O.Z., Kolesnikova O.A., Schirtz T., Vysokikh M.Y., Mager-Heckel A.M., Lombes A., Boucheham A., Krasheninnikov I.A., Martin R.P., Entelis N., Tarassov I. Correction of the consequences of mitochondrial 3243A&gt;G mutation in the MT-TL1 gene causing the MELAS syndrome by tRNA import into mitochondria // Nucleic Acids Res. 2011. Vol. 39. N 18. P. 8173—8186.</mixed-citation><mixed-citation xml:lang="en">Karicheva O.Z., Kolesnikova O.A., Schirtz T., Vysokikh M.Y., Mager-Heckel A.M., Lombes A., Boucheham A., Krasheninnikov I.A., Martin R.P., Entelis N., Tarassov I. Correction of the consequences of mitochondrial 3243A&gt;G mutation in the MT-TL1 gene causing the MELAS syndrome by tRNA import into mitochondria // Nucleic Acids Res. 2011. Vol. 39. N 18. P. 8173—8186.</mixed-citation></citation-alternatives></ref><ref id="cit25"><label>25</label><citation-alternatives><mixed-citation xml:lang="ru">Kolesnikova O., Kazakova H., Comte C., Steinberg S., Kamenski P., Martin R.P., Tarassov I., Entelis N. Selection of RNA aptamers imported into yeast and human mitochondria // RNA. 2010. Vol. 16. N 5. P. 926—941.</mixed-citation><mixed-citation xml:lang="en">Kolesnikova O., Kazakova H., Comte C., Steinberg S., Kamenski P., Martin R.P., Tarassov I., Entelis N. Selection of RNA aptamers imported into yeast and human mitochondria // RNA. 2010. Vol. 16. N 5. P. 926—941.</mixed-citation></citation-alternatives></ref><ref id="cit26"><label>26</label><citation-alternatives><mixed-citation xml:lang="ru">Comte C., Tonin Y., Heckel-Mager A.M., Boucheham A., Smirnov A., Aure K., Lombes A., Martin R.P., Entelis N., Tarassov I. Mitochondrial targeting of recombinant RNAs modulates the level of a heteroplasmic mutation in human mitochondrial DNA associated with Kearns Sayre Syndrome // Nucleic Acids Res. 2013. Vol. 41. N 1. P. 418—433.</mixed-citation><mixed-citation xml:lang="en">Comte C., Tonin Y., Heckel-Mager A.M., Boucheham A., Smirnov A., Aure K., Lombes A., Martin R.P., Entelis N., Tarassov I. Mitochondrial targeting of recombinant RNAs modulates the level of a heteroplasmic mutation in human mitochondrial DNA associated with Kearns Sayre Syndrome // Nucleic Acids Res. 2013. Vol. 41. N 1. P. 418—433.</mixed-citation></citation-alternatives></ref><ref id="cit27"><label>27</label><citation-alternatives><mixed-citation xml:lang="ru">Tonin Y., Heckel A.M., Vysokikh M., Dovydenko I., Meschaninova M., Rotig A., Munnich A., Venyaminova A., Tarassov I., Entelis N. Modeling of antigenomic therapy of mitochondrial diseases by mitochondrially addressed RNA targeting a pathogenic point mutation in mitochondrial DNA // J. Biol. Chem. 2014. Vol. 289. N 19. P. 13323—13334.</mixed-citation><mixed-citation xml:lang="en">Tonin Y., Heckel A.M., Vysokikh M., Dovydenko I., Meschaninova M., Rotig A., Munnich A., Venyaminova A., Tarassov I., Entelis N. Modeling of antigenomic therapy of mitochondrial diseases by mitochondrially addressed RNA targeting a pathogenic point mutation in mitochondrial DNA // J. Biol. Chem. 2014. Vol. 289. N 19. P. 13323—13334.</mixed-citation></citation-alternatives></ref></ref-list><fn-group><fn fn-type="conflict"><p>The authors declare that there are no conflicts of interest present.</p></fn></fn-group></back></article>
