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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">nodgo</journal-id><journal-title-group><journal-title xml:lang="ru">Российский журнал детской гематологии и онкологии (РЖДГиО)</journal-title><trans-title-group xml:lang="en"><trans-title>Russian Journal of Pediatric Hematology and Oncology</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">2311-1267</issn><issn pub-type="epub">2413-5496</issn><publisher><publisher-name>LTD “Graphica”</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.21682/2311-1267-2019-6-2-54-60</article-id><article-id custom-type="elpub" pub-id-type="custom">nodgo-497</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>ОБЗОРЫ ЛИТЕРАТУРЫ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>LITERATURE REVIEWS</subject></subj-group></article-categories><title-group><article-title>ALK и нейробластома: от молекулярной генетики до клиники</article-title><trans-title-group xml:lang="en"><trans-title>ALK and neuroblastoma: from molecular genetics to clinics</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-5626-218X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Андреева</surname><given-names>Н. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Andreeva</surname><given-names>N. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Наталья Александровна Андреева </p><p>Врач - детский онколог отделения клинической онкологии </p><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>Pediatric Oncologist, Department of Clinical Oncology Dmitry Rogachev </p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">andreeva793@bk.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-1308-8622</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Друй</surname><given-names>А. Е.</given-names></name><name name-style="western" xml:lang="en"><surname>Druy</surname><given-names>A. E.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Кандидат медицинских наук, старший научный сотрудник лаборатории цитогенетики и молекулярной генетики</p><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Senior Researcher, Laboratory of Cytogenetics and Molecular Genetics </p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">DrDrui@yandex.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-3767-4477</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Шаманская</surname><given-names>Т. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Shamanskaya</surname><given-names>T. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Кандидат медицинских наук, ученый секретарь Института онкологии, радиологии и ядерной медицины</p><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Academic Secretary of the Institute of Oncology, Radiology and Nuclear Medicine </p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">shamanskaya.tatyana@gmail.com</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-3704-8783</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Качанов</surname><given-names>Д. Ю.</given-names></name><name name-style="western" xml:lang="en"><surname>Kachanov</surname><given-names>D. Yu.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Доктор медицинских наук, заведующий отделением клинической онкологии, заместитель директора Института онкологии, радиологии и ядерной медицины </p><p>SPIN-код: 98785540</p><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>Dr. of Sci. (Med.), Head of the Department of Clinical Oncology and Department of Embryonic Tumor Research &amp; Deputy Director of the Institute of Oncology, Radiology and Nuclear Medicine </p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">Denis.Kachanov@fcchomoscow.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-6131-1783</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Варфоломеева</surname><given-names>С. Р.</given-names></name><name name-style="western" xml:lang="en"><surname>Varfolomeeva</surname><given-names>S. R.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Доктор медицинских наук, профессор, заместитель генерального директора – директор Института управления и трансляционной медицины НМИЦ ДГОИ им. Дмитрия Рогачева, исполнительный директор РОО НОДГО</p><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>Dr. of Sci. (Med.), Professor, Deputy General Director – Director of the Institute of Management and Translational Medicine at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia, Executive Director of Regional Public Organization National Society of Pediatric Hematologists and Oncologists</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">clinoncology@gmail.com</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2019</year></pub-date><pub-date pub-type="epub"><day>23</day><month>04</month><year>2019</year></pub-date><volume>6</volume><issue>2</issue><fpage>54</fpage><lpage>60</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Андреева Н.А., Друй А.Е., Шаманская Т.В., Качанов Д.Ю., Варфоломеева С.Р., 2019</copyright-statement><copyright-year>2019</copyright-year><copyright-holder xml:lang="ru">Андреева Н.А., Друй А.Е., Шаманская Т.В., Качанов Д.Ю., Варфоломеева С.Р.</copyright-holder><copyright-holder xml:lang="en">Andreeva N.A., Druy A.E., Shamanskaya T.V., Kachanov D.Y., Varfolomeeva S.R.</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://journal.nodgo.org/jour/article/view/497">https://journal.nodgo.org/jour/article/view/497</self-uri><abstract><p>Нейробластома (НБ) – самая частая экстракраниальная эмбриональная опухоль у детей с разнообразными молекулярнобиологическими и клиническими характеристиками. Единого молекулярногенетического механизма, участвующего в патогенезе НБ нет, что и обусловливает ее гетерогенность. Патогенетически важным событием в развитии НБ являются аберрации гена ALK (Anaplastic lymphoma kinase), которые обнаруживаются у 70 % больных с семейной формой НБ и у 7– 10 % пациентов со спорадическими случаями. Онкоген ALK кодирует одноименный рецептор, экспрессирующийся на мембране клеток центральной и периферической нервной системы, который находится в активированном состоянии при НБ. Описано негативное влияние аномалий гена ALK на прогноз при НБ у пациентов разных групп риска. Аберрации гена ALK чаще выявляются при рецидиве и рефрактерном течении болезни. Ввиду своей тканеспецифичности белок ALK является идеальной мишенью для таргетной терапии. В данной статье представлен литературный обзор роли ALK при НБ.</p></abstract><trans-abstract xml:lang="en"><p>Neuroblastoma (NB) is the most common extracranial embryonic tumor in children with a variety of molecular biological and clinical characteristics. There is no single molecular genetic mechanism involved in the pathogenesis of NB, which determines its heterogeneity. Pathogenetically important event in the development of NB are aberrations of ALK gene (Anaplastic lymphoma kinase), which are found in 70 % of patients with familial form of NB and in 7– 10 % of patients with sporadic cases. ALK oncogene encodes a receptor of the same name, expressed on the membrane of cells of the central and peripheral nervous system, which is in the activated state in NB. The negative effect of ALK gene anomalies on the prognosis in patients with different risk groups of NB is described. ALK gene aberrations are more often detected duringrelapse and refractory course of the disease. Because of its tissue specificity, ALK protein is an ideal target for targeted therapy. This article presents a literature review of the role of ALK in NB.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>нейробластома</kwd><kwd>дети</kwd><kwd>онкоген ALK</kwd><kwd>миссенсмутации ALK</kwd><kwd>высокопроизводительное секвенирование</kwd><kwd>ALK-ингибиторы</kwd></kwd-group><kwd-group xml:lang="en"><kwd>neuroblastoma</kwd><kwd>children</kwd><kwd>ALK oncogene</kwd><kwd>ALK missense mutations</kwd><kwd>highthroughput sequencing</kwd><kwd>ALK inhibitors</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">Schulte J.H., Schulte S., Heukamp L.C., Astrahantseff K., Stephan H., Fischer M., Schramm A., Eggert A. Targeted therapy for neuroblastoma: ALK inhibitors. 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