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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">kpccz</journal-id><journal-title-group><journal-title xml:lang="ru">Комплексные проблемы сердечно-сосудистых заболеваний</journal-title><trans-title-group xml:lang="en"><trans-title>Complex Issues of Cardiovascular Diseases</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">2306-1278</issn><issn pub-type="epub">2587-9537</issn><publisher><publisher-name>Federal State Budgetary Institution “Research Institute for Complex Issues of Cardiovascular Diseases”</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.17802/2306-1278-2019-8-3-85-95</article-id><article-id custom-type="elpub" pub-id-type="custom">kpccz-600</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>АНАЛИТИЧЕСКИЙ ОБЗОР</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>ANALYTICAL REVIEW</subject></subj-group></article-categories><title-group><article-title>ПОСТТРАНСКРИПЦИОННОЕ РЕГУЛИРОВАНИЕ В РАЗВИТИИ ВРОЖДЁННЫХ ПОРОКОВ СЕРДЦА: ЗНАЧЕНИЕ микроРНК</article-title><trans-title-group xml:lang="en"><trans-title>POSTTRANSCRIPTIONAL REGULATION IN CONGENITAL HEART DISEASE: THE ROLE OF miRNA</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-3002-2863</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Понасенко</surname><given-names>А. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Ponasenko</surname><given-names>A. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Понасенко Анастасия Валериевна, кандидат медицинских наук, заведующая лабораторией геномной медицины отдела экспериментальной и клинической кардиологии</p><p>ул. Сосновый бульвар, 6, Кемерово</p></bio><bio xml:lang="en"><p>Ponasenko Anastasia V., PhD, Head of the Laboratory of Genomic Medicine, Department of Experimental and Clinical Cardiology</p><p>6, Sosnoviy Blvd, Kemerovo, 650002 </p></bio><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-4467-8732</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Цепокина</surname><given-names>А. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Tsepokina</surname><given-names>A. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Цепокина Анна Викторовна, младший научный сотрудник лаборатории геномной медицины отдела экспериментальной и клинической кардиологии</p><p>ул. Сосновый бульвар, 6, Кемерово</p></bio><bio xml:lang="en"><p>Tsepokina Anna V., researcher assistant at the Laboratory of Genomic Medicine, Department of Experimental and Clinical Cardiology</p><p>6, Sosnoviy Blvd, Kemerovo, 650002 </p></bio><email xlink:type="simple">cepoav1991@gmail.com</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru">Федеральное государственное бюджетное научное учреждение «Научно-исследовательский институт комплексных проблем сердечно-сосудистых заболеваний»<country>Россия</country></aff><aff xml:lang="en">Federal State Budgetary Institution “Research Institute for Complex Issues of Cardiovascular Diseases”<country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2019</year></pub-date><pub-date pub-type="epub"><day>26</day><month>09</month><year>2019</year></pub-date><volume>8</volume><issue>3</issue><fpage>85</fpage><lpage>95</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Понасенко А.В., Цепокина А.В., 2019</copyright-statement><copyright-year>2019</copyright-year><copyright-holder xml:lang="ru">Понасенко А.В., Цепокина А.В.</copyright-holder><copyright-holder xml:lang="en">Ponasenko A.V., Tsepokina A.V.</copyright-holder><license 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://www.nii-kpssz.com/jour/article/view/600">https://www.nii-kpssz.com/jour/article/view/600</self-uri><abstract><p>Врожденные пороки сердца являются наиболее распространённой аномалией плода и одной из главных причин детской инвалидизации и смертности. Врожденные пороки сердца представляет собой гетерогенную группу сердечных аномалий, включающую дефект предсердной перегородки, дефекты клапанного аппарата и аномалии оттока. Генетическая, эпигенетическая или экологическая основа врожденного порока сердца в каждом конкретном эпизоде заболевания остается недостаточно понятной. Вероятнее всего, механизм формирования порока является многофакторным и полиэтиологичным. Тем не менее, разрабатываемые новые современные технологии генетической диагностики, включая полиморфизм замен одного нуклеотида, варианты числа копий, секвенирование следующего поколения, ускоряет обнаружение генетических причин аномалий сердца. Недавние исследования предполагают роль малых некодирующих РНК (микроРНК) в патогенезе врожденных пороков сердца. Установлено, что микроРНК координируют развитие сердца и стимулируют патологические процессы в нем, включая фиброз или гипертрофию и нарушение ангиогенеза. Таким образом, возможность изучения микроРНК и путей их влияния при формировании разных пороков сердца демонстрирует большой потенциал в качестве терапевтических мишеней в регенеративной медицине. В этом обзоре мы представляем прошлые и недавние генетические открытия, даём обзор функции микроРНК, сигнальные пути, через которые реализуются их функции и выясняем их роль в отдельных врожденных пороках сердца.</p></abstract><trans-abstract xml:lang="en"><p>Congenital heart disease is the most common fetal abnormality resulting in high pediatric disability and mortality. Congenital heart disease is a heterogeneous group of cardiac abnormalities including atrial septal defect, valvular defects and cardiac outflow tract anomalies. Genetic, epigenetic and ecological factors leading to the development of congenital heart defects in each particular case remain poorly understood. Nevertheless, multifactorial and polygenic mechanisms underlying the disease may be suggested. Moreover, advanced genetic technologies including single nucleotide polymorphism testing, copy number variation and next-generation sequencing ensure early detection of genetic causes of heart abnormalities. Recent studies suggested the contributing role of small non-coding RNA (miRNA) in the pathogenesis of congenital heart defects. miRNA is known to coordinate the development of heart and stimulate such pathological processes like fibrosis, hypertrophy and impaired angiogenesis. Thus, the study of miRNA and its impact on the pathogenesis of various heart diseases has demonstrated its promising potential for therapeutic targets in regenerative medicine. The review presents recent genetic findings, miRNA functions, signaling pathways and evidences on its role in the development of certain congenital heart defects.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>Врождённые пороки сердца</kwd><kwd>Эмбриогенез</kwd><kwd>микроРНК</kwd></kwd-group><kwd-group xml:lang="en"><kwd>Congenital heart diseases</kwd><kwd>Embryogenesis</kwd><kwd>miRNA</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">Шабалдин А.В., Глебова Л.А., Бачина А.В., Счастливцев Е.Л., Потапов В.П. Особенности эпидемиологии врожденных пороков сердца у детей г. Кемерово как крупного промышленного центра. Комплексные проблемы сердечно-сосудистых заболеваний. 2014;(4):38-46. https://doi.org/10.17802/2306-1278-2014-4-38-46</mixed-citation><mixed-citation xml:lang="en">Shabaldin A.V., Glebova L.A., Bachina A.V., Schastlivcev E.L., Potapov V.P. 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