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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="review-article" dtd-version="1.2" xml:lang="en"><front><journal-meta><journal-id journal-id-type="publisher-id">Terapevticheskii arkhiv</journal-id><journal-title-group><journal-title xml:lang="en">Terapevticheskii arkhiv</journal-title><trans-title-group xml:lang="ru"><trans-title>Терапевтический архив</trans-title></trans-title-group></journal-title-group><issn publication-format="print">0040-3660</issn><issn publication-format="electronic">2309-5342</issn><publisher><publisher-name xml:lang="en">LLC Obyedinennaya Redaktsiya</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="publisher-id">634101</article-id><article-id pub-id-type="doi">10.26442/00403660.2024.06.202732</article-id><article-categories><subj-group subj-group-type="toc-heading" xml:lang="en"><subject>History of medicine</subject></subj-group><subj-group subj-group-type="toc-heading" xml:lang="ru"><subject>История медицины</subject></subj-group><subj-group subj-group-type="article-type"><subject>Review Article</subject></subj-group></article-categories><title-group><article-title xml:lang="en">History of the study of amyloidosis: from the Rokitansky’s theory to the present day</article-title><trans-title-group xml:lang="ru"><trans-title>История изучения амилоидоза: от теории Рокитанского до настоящих дней</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-4260-0226</contrib-id><name-alternatives><name xml:lang="en"><surname>Rameev</surname><given-names>Vilen V.</given-names></name><name xml:lang="ru"><surname>Рамеев</surname><given-names>Вилен Вилевич</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="ru"><p>д-р мед. наук, проф. каф. внутренних, профессиональных болезней и ревматологии Института клинической медицины им. Н.В. Склифосовского</p></bio><email>vvrameev@mail.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-1166-7308</contrib-id><name-alternatives><name xml:lang="en"><surname>Lysenko</surname><given-names>Lidia V.</given-names></name><name xml:lang="ru"><surname>Лысенко</surname><given-names>Лидия Владимировна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="ru"><p>д-р мед. наук, проф. каф. внутренних, профессиональных болезней и ревматологии Института клинической медицины им. Н.В. Склифосовского</p></bio><email>vvrameev@mail.ru</email><xref ref-type="aff" rid="aff1"/></contrib></contrib-group><aff-alternatives id="aff1"><aff><institution xml:lang="en">Sechenov First Moscow State Medical University (Sechenov University)</institution></aff><aff><institution xml:lang="ru">ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет)</institution></aff></aff-alternatives><pub-date date-type="pub" iso-8601-date="2024-07-07" publication-format="electronic"><day>07</day><month>07</month><year>2024</year></pub-date><volume>96</volume><issue>6</issue><issue-title xml:lang="en">Issues of nephrology</issue-title><issue-title xml:lang="ru">Вопросы нефрологии</issue-title><fpage>635</fpage><lpage>640</lpage><history><date date-type="received" iso-8601-date="2024-07-07"><day>07</day><month>07</month><year>2024</year></date><date date-type="accepted" iso-8601-date="2024-07-07"><day>07</day><month>07</month><year>2024</year></date></history><permissions><copyright-statement xml:lang="en">Copyright ©; 2024, Consilium Medicum</copyright-statement><copyright-statement xml:lang="ru">Copyright ©; 2024, ООО "Консилиум Медикум"</copyright-statement><copyright-year>2024</copyright-year><copyright-holder xml:lang="en">Consilium Medicum</copyright-holder><copyright-holder xml:lang="ru">ООО "Консилиум Медикум"</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-nc-sa/4.0</ali:license_ref></license></permissions><self-uri xlink:href="https://ter-arkhiv.ru/0040-3660/article/view/634101">https://ter-arkhiv.ru/0040-3660/article/view/634101</self-uri><abstract xml:lang="en"><p>In the history of amyloidosis studying the concept of liquids dyscrasia has been predominated and finally it is resulted in accepting a serum protein-precursor as a leading amyloidogenic factor in the disease pathogenesis. Consequently basic diagnostic and treatment strategy was aimed to find and eliminate this protein from the blood and this approach evidenced high effectiveness in most frequent AA and AL-amyloidosis characterized with anomaly high levels of precursors in the blood. At the same time there are less frequent and slower progressing inheritant forms of systemic amyloidosis including transthyretin induced, which are less depending on amyloidogenecity of amyloid precursor and because of that, in example, the effectiveness of transthyretin stabilizers or blockers of its synthesis is limited comparing with the precursor elimination in AA or AL. Developed in the middle of XX century a theory of local synthesis by macrophages is more preferable to describe the pathogenesis of these forms. And modern proteome analysis using give rise to confirm the key meaning of macrophage in the amyloidogenesis and proves necessity to know deeply mechanisms of macrophagial autophagia – basic tool of maintaining intracellular protein homeostasis. That is why it is difficult to hope on high effectiveness of chemical amyloid solvents in vivo, which being under macrophages regulation never could realize its chemical activities.</p></abstract><trans-abstract xml:lang="ru"><p>В истории изучения амилоидоза преимущественно доминировала концепция дискразии жидкостей – диспротеиноза, которая вылилась в конечном итоге в признание ведущей роли в генезе заболевания амилоидогенности белка-предшественника, выявление и элиминация которого из крови составляют основную диагностическую и терапевтическую задачу в клинике. Данный подход оказался высокоэффективным в отношении наиболее распространенных форм амилоидоза – вторичного и первичного – с аномально высокими концентрациями белков-предшественников в крови. Менее распространены и медленнее прогрессируют наследственные формы амилоидоза, в том числе транстиретинового, которые меньше зависят от амилоидогенности белка-предшественника, а применение, например, стабилизаторов транстиретина или блокада его синтеза при транстиретиновом амилоидозе имеет ограниченную эффективность. Для объяснения патогенеза приведенных форм более приемлема концепция локального макрофагального синтеза амилоида, которая развивается с середины ХХ в. Современные методы протеомного анализа позволяют подтвердить ключевую роль макрофага в амилоидогенезе и необходимость тщательного исследования механизмов макрофагальной аутофагии – главного инструмента поддержания белкового гомеостаза в клетке. Соответственно, не следует ожидать высокой эффективности и от химического растворения амилоида <italic>in vivo</italic>, потому что активность химической субстанции всегда будет контролироваться макрофагом.</p></trans-abstract><kwd-group xml:lang="en"><kwd>amyloidosis</kwd><kwd>dyscrasia</kwd><kwd>K. Rokitansky</kwd><kwd>lardaceous disease</kwd><kwd>R. Virchow</kwd><kwd>congo red</kwd><kwd>macrophage</kwd><kwd>autophagy</kwd><kwd>proteomic analysis</kwd><kwd>chaperones</kwd><kwd>ubiquitine proteasome</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>амилоидоз</kwd><kwd>дискразия, K. Рокитанский</kwd><kwd>сальная болезнь</kwd><kwd>P. Вирхов</kwd><kwd>конго красный</kwd><kwd>макрофаг</kwd><kwd>аутофагия</kwd><kwd>протеомный анализ</kwd><kwd>шапероны</kwd><kwd>убиквитиновая протеасома</kwd></kwd-group><funding-group/></article-meta></front><body></body><back><ref-list><ref id="B1"><label>1.</label><mixed-citation>Cohen AS, Calkins E. 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