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<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">ssmu</journal-id><journal-title-group><journal-title xml:lang="ru">Бюллетень сибирской медицины</journal-title><trans-title-group xml:lang="en"><trans-title>Bulletin of Siberian Medicine</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">1682-0363</issn><issn pub-type="epub">1819-3684</issn><publisher><publisher-name>Siberian State Medical University, the Ministry of Healthcare of the Russian Federation</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.20538/1682-0363-2013-3-5-17</article-id><article-id custom-type="elpub" pub-id-type="custom">ssmu-333</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>ПЕРЕДОВАЯ СТАТЬЯ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>EDITORIAL</subject></subj-group></article-categories><title-group><article-title>МИТОХОНДРИАЛЬНЫЙ БЕЛКОВЫЙ ПРОФИЛЬ И ЕГО РОЛЬ В ПАТОЛОГИЧЕСКИХ ПРОЦЕССАХ</article-title><trans-title-group xml:lang="en"><trans-title>MITOCHONDRIAL PROTEIN PROFILE AND ITS ROLE IN PATHOLOGIC PROCESSES</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>Костерина</surname><given-names>Е. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Kosterina</surname><given-names>Ye. A.</given-names></name></name-alternatives><bio xml:lang="ru"/><email xlink:type="simple">mpatrushev@innopark.kantiana.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>Козенков</surname><given-names>И. И.</given-names></name><name name-style="western" xml:lang="en"><surname>Kozenkov</surname><given-names>I. I.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Младший научны сотрудник лаборатории геномных и протеомных исследований </p></bio><email xlink:type="simple">mpatrushev@innopark.kantiana.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>Касымов</surname><given-names>В. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Kasymov</surname><given-names>V. A.</given-names></name></name-alternatives><bio xml:lang="ru"/><email xlink:type="simple">mpatrushev@innopark.kantiana.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>П. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Kamensky</surname><given-names>P. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Кандидат биологических наук,  старший научный сотрудник лаборатории геномных и протеомных исследований </p></bio><email xlink:type="simple">mpatrushev@innopark.kantiana.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>Доминова</surname><given-names>И. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Dominova</surname><given-names>I. N.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Младший научныйсотрудник лаборатории геномных и протеомных исследований</p></bio><email xlink:type="simple">mpatrushev@innopark.kantiana.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>Королёва</surname><given-names>Ю. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Korolyova</surname><given-names>Yu. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Младший научныйсотрудник лаборатории геномных и протеомных исследований</p></bio><email xlink:type="simple">mpatrushev@innopark.kantiana.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>Патрушева</surname><given-names>В. Е.</given-names></name><name name-style="western" xml:lang="en"><surname>Patrusheva</surname><given-names>V. Ye.</given-names></name></name-alternatives><bio xml:lang="ru"/><email xlink:type="simple">mpatrushev@innopark.kantiana.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>Богачев</surname><given-names>Р. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Bogachev</surname><given-names>R. S.</given-names></name></name-alternatives><bio xml:lang="ru"/><email xlink:type="simple">mpatrushev@innopark.kantiana.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>Литвинова</surname><given-names>Л. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Litvinova</surname><given-names>L. S.</given-names></name></name-alternatives><bio xml:lang="ru"/><email xlink:type="simple">mpatrushev@innopark.kantiana.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>Бабак</surname><given-names>С. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Babak</surname><given-names>S. V.</given-names></name></name-alternatives><bio xml:lang="ru"/><email xlink:type="simple">mpatrushev@innopark.kantiana.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>Моисеева</surname><given-names>Е. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Moiseeva</surname><given-names>Ye. M.</given-names></name></name-alternatives><bio xml:lang="ru"/><email xlink:type="simple">mpatrushev@innopark.kantiana.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>Богданов</surname><given-names>Е. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Bogdanov</surname><given-names>Ye. A.</given-names></name></name-alternatives><bio xml:lang="ru"/><email xlink:type="simple">mpatrushev@innopark.kantiana.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>Мухортова</surname><given-names>О. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Mukhortova</surname><given-names>O. A.</given-names></name></name-alternatives><bio xml:lang="ru"/><email xlink:type="simple">mpatrushev@innopark.kantiana.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>Вавилина</surname><given-names>Я. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Vavilina</surname><given-names>Ya. S.</given-names></name></name-alternatives><bio xml:lang="ru"/><email xlink:type="simple">mpatrushev@innopark.kantiana.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>Михальченкова</surname><given-names>Т. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Mikhalchenkova</surname><given-names>T. A.</given-names></name></name-alternatives><bio xml:lang="ru"/><email xlink:type="simple">mpatrushev@innopark.kantiana.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>Патрушев</surname><given-names>М. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Patrushev</surname><given-names>M. V.</given-names></name></name-alternatives><bio xml:lang="ru"/><bio xml:lang="en"/><email xlink:type="simple">mpatrushev@innopark.kantiana.ru</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Балтийский федеральный университет имени И. Канта, Калининград</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Immanuel Kant Baltic Federal University, Kaliningrad</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2013</year></pub-date><pub-date pub-type="epub"><day>28</day><month>06</month><year>2013</year></pub-date><volume>12</volume><issue>3</issue><fpage>5</fpage><lpage>17</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Костерина Е.А., Козенков И.И., Касымов В.А., Каменский П.А., Доминова И.Н., Королёва Ю.А., Патрушева В.Е., Богачев Р.С., Литвинова Л.С., Бабак С.В., Моисеева Е.М., Богданов Е.А., Мухортова О.А., Вавилина Я.С., Михальченкова Т.А., Патрушев М.В., 2013</copyright-statement><copyright-year>2013</copyright-year><copyright-holder xml:lang="ru">Костерина Е.А., Козенков И.И., Касымов В.А., Каменский П.А., Доминова И.Н., Королёва Ю.А., Патрушева В.Е., Богачев Р.С., Литвинова Л.С., Бабак С.В., Моисеева Е.М., Богданов Е.А., Мухортова О.А., Вавилина Я.С., Михальченкова Т.А., Патрушев М.В.</copyright-holder><copyright-holder xml:lang="en">Kosterina Y.A., Kozenkov I.I., Kasymov V.A., Kamensky P.A., Dominova I.N., Korolyova Y.A., Patrusheva V.Y., Bogachev R.S., Litvinova L.S., Babak S.V., Moiseeva Y.M., Bogdanov Y.A., Mukhortova O.A., Vavilina Y.S., Mikhalchenkova T.A., Patrushev M.V.</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://bulletin.ssmu.ru/jour/article/view/333">https://bulletin.ssmu.ru/jour/article/view/333</self-uri><abstract><p>Собственная ДНК митохондрий кодирует лишь 13 полипептидов – субъединиц электронтранспортной цепи. Большинство же белков импортируются в митохондрии посредством различных механизмов. Последние исследования митохондриального протеома продемонстрировали истинные размеры и сложную функциональную организацию митохондриальных белковых профилей. Для протеомного профилирования митохондрий применяются различные технологии, которые в сумме представляют результаты, помогающие понять молекулярные механизмы функционирования органелл и выявить нарушения, приводящие к различным заболеваниям. В данном обзоре рассмотрены и проанализированны последние данные в области протеомики митохондрий и затронуты некоторые аспекты молекулярного патогенеза заболеваний дыхательной цепи.</p></abstract><trans-abstract xml:lang="en"><p>Mitochondria import hundreds of different precursor proteins from the cytosol, and only 13 proteins are encoded by mtDNA itself. Recent investigations demonstrated real size of mitochondrial proteome and complexity of their functions There are many methods using for mitochondrial proteome profiling, that help to understand a molecular mechanisms of mitochondrial functions and identify the causes of disruptions that lead to different disorders. In this review we discuss a recent data in the field of mitochondrial proteomics.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>митохондриальный протеом</kwd><kwd>импорт белков</kwd><kwd>компоненты дыхательной цепи</kwd><kwd>митохондриальная ДНК</kwd><kwd>заболевания дыхательной цепи</kwd></kwd-group><kwd-group xml:lang="en"><kwd>mitochondrial proteome</kwd><kwd>protein import</kwd><kwd>respiratory chain proteins</kwd><kwd>mitochondrial DNA</kwd><kwd>respiratory chain disorders</kwd></kwd-group></article-meta></front><back><ref-list><title>References</title><ref id="cit1"><label>1</label><citation-alternatives><mixed-citation xml:lang="ru">Anderson S., Bankier A.T., Barrell B.G. et al. Sequence and organization of the human mitochondrial genome // Nature. 1981. 290. P. 457–465.</mixed-citation><mixed-citation xml:lang="en">Anderson S., Bankier A.T., Barrell B.G., de Bruijn M.H., Coulson A.R. et al. Sequence and organization of the human mitochondrial genome. 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