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<article 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" xmlns:ali="http://www.niso.org/schemas/ali/1.0/" article-type="research-article" dtd-version="1.2" xml:lang="en"><front><journal-meta><journal-id journal-id-type="publisher-id">Acta Naturae</journal-id><journal-title-group><journal-title xml:lang="en">Acta Naturae</journal-title><trans-title-group xml:lang="ru"><trans-title>Acta Naturae</trans-title></trans-title-group></journal-title-group><issn publication-format="print">2075-8251</issn><publisher><publisher-name xml:lang="en">Acta Naturae Ltd</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="publisher-id">10387</article-id><article-id pub-id-type="doi">10.32607/20758251-2017-9-2-34-46</article-id><article-categories><subj-group subj-group-type="toc-heading" xml:lang="en"><subject>Reviews</subject></subj-group><subj-group subj-group-type="toc-heading" xml:lang="ru"><subject>Обзоры</subject></subj-group><subj-group subj-group-type="article-type"><subject>Research Article</subject></subj-group></article-categories><title-group><article-title xml:lang="en">Huntington’s Disease: Calcium Dyshomeostasis and Pathology Models</article-title><trans-title-group xml:lang="ru"><trans-title>Болезнь Хантингтона: нарушения кальциевой сигнализации и модели для изучения развития патологии</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Kolobkova</surname><given-names>Yu. A.</given-names></name><name xml:lang="ru"><surname>Колобкова</surname><given-names>Ю. A.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><email>evkazn@incras.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Vigont</surname><given-names>V. A.</given-names></name><name xml:lang="ru"><surname>Вигонт</surname><given-names>В. A.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><email>evkazn@incras.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Shalygin</surname><given-names>A. V.</given-names></name><name xml:lang="ru"><surname>Шалыгин</surname><given-names>A. В.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><email>evkazn@incras.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Kaznacheyeva</surname><given-names>E. V.</given-names></name><name xml:lang="ru"><surname>Казначеева</surname><given-names>E. В.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><email>evkazn@incras.ru</email><xref ref-type="aff" rid="aff1"/></contrib></contrib-group><aff-alternatives id="aff1"><aff><institution xml:lang="en">Institute of Сytology of the Russian Academy of Sciences</institution></aff><aff><institution xml:lang="ru">Институт цитологии РАН</institution></aff></aff-alternatives><pub-date date-type="pub" iso-8601-date="2017-06-15" publication-format="electronic"><day>15</day><month>06</month><year>2017</year></pub-date><volume>9</volume><issue>2</issue><issue-title xml:lang="en">VOL 9, NO2 (2017)</issue-title><issue-title xml:lang="ru">ТОМ 9, №2 (2017)</issue-title><fpage>34</fpage><lpage>46</lpage><history><date date-type="received" iso-8601-date="2020-01-17"><day>17</day><month>01</month><year>2020</year></date></history><permissions><copyright-statement xml:lang="en">Copyright ©; 2017, Kolobkova Y.A., Vigont V.A., Shalygin A.V., Kaznacheyeva E.V.</copyright-statement><copyright-statement xml:lang="ru">Copyright ©; 2017, Колобкова Ю.A., Вигонт В.A., Шалыгин A.В., Казначеева E.В.</copyright-statement><copyright-year>2017</copyright-year><copyright-holder xml:lang="en">Kolobkova Y.A., Vigont V.A., Shalygin A.V., Kaznacheyeva E.V.</copyright-holder><copyright-holder xml:lang="ru">Колобкова Ю.A., Вигонт В.A., Шалыгин A.В., Казначеева E.В.</copyright-holder><ali:free_to_read xmlns:ali="http://www.niso.org/schemas/ali/1.0/"/><license><ali:license_ref xmlns:ali="http://www.niso.org/schemas/ali/1.0/">https://creativecommons.org/licenses/by/4.0</ali:license_ref></license></permissions><self-uri xlink:href="https://actanaturae.ru/2075-8251/article/view/10387">https://actanaturae.ru/2075-8251/article/view/10387</self-uri><abstract xml:lang="en"><p>Huntington’s disease (HD) is a severe inherited neurodegenerative disorder characterized by motor dysfunction, cognitive decline, and mental impairment. At the molecular level, HD is caused by a mutation in the first exon of the gene encoding the huntingtin protein. The mutation results in an expanded polyglutamine tract at the N-terminus of the huntingtin protein, causing the neurodegenerative pathology. Calcium dyshomeostasis is believed to be one of the main causes of the disease, which underlies the great interest in the problem among experts in molecular physiology. Recent studies have focused on the development of animal and insect HD models, as well as patient-specific induced pluripotent stem cells (HD-iPSCs), to simulate the disease’s progression. Despite a sesquicentennial history of HD studies, the issues of diagnosis and manifestation of the disease have remained topical. The present review addresses these issues.</p></abstract><trans-abstract xml:lang="ru"><p>Болезнь Хантингтона (БХ) - это тяжелое наследственное нейродегенеративное заболевание, характеризующееся моторной дисфункцией, снижением когнитивных способностей и психическими расстройствами. БХ обусловлена мутацией в первом экзоне гена, кодирующего белок хантингтин, которая приводит к увеличению полиглутаминового тракта в N-концевой области хантингтина и развитию тяжелой нейродегенеративной патологии. Есть все основания рассматривать нарушение кальциевого гомеостаза в качестве одной из ключевых причин развития патологии, что объясняет повышенный интерес к данной проблеме специалистов в области молекулярной физиологии. Большое внимание уделяется созданию животных моделей и пациент-специфичных индуцированных плюрипотентных стволовых клеток (HDiPSC), имитирующих развитие заболевания. Несмотря на полуторавековую историю исследований БХ, необходимо констатировать, что проблемы манифестации и диагностики данной патологии актуальны и по сей день. Перечисленные проблемы нашли отражение в настоящем обзоре.</p></trans-abstract><kwd-group xml:lang="en"><kwd>calcium</kwd><kwd>induced pluripotent stem cells (HD-iPSCs)</kwd><kwd>huntington’s disease</kwd><kwd>neurodegeneration</kwd><kwd>huntingtin</kwd><kwd>SOC</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>болезнь Хантингтона</kwd><kwd>кальций</kwd><kwd>нейродегенерация</kwd><kwd>хантингтин</kwd><kwd>индуцированные плюрипотентные стволовые клетки (HDiPSC)</kwd><kwd>SOC</kwd></kwd-group><funding-group><funding-statement xml:lang="en">This work was supported by the Russian Science Foundation, grant No. 14-14-00720 (EVK), the Russian Academy of Sciences Presidium Programme Molecular and Cellular Biology (YuAK), and scholarships of the President of the Russian Federation (VAV and AVSh).</funding-statement><funding-statement xml:lang="ru">Работа поддержана грантом РНФ №14-14-00720 (ЕВК), Программой Президиума РАН «Молекулярная и клеточная биология» (ЮАК), стипендиями Президента РФ (ВАВ и АВШ).</funding-statement></funding-group></article-meta></front><body></body><back><ref-list><ref id="B1"><label>1.</label><mixed-citation>[1] Huntington G. // Med. 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