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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">adair</journal-id><journal-title-group><journal-title xml:lang="ru">Аллергология и Иммунология в Педиатрии</journal-title><trans-title-group xml:lang="en"><trans-title>Allergology and Immunology in Paediatrics</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">2500-1175</issn><issn pub-type="epub">2712-7958</issn><publisher><publisher-name>Ассоциация детских аллергологов и иммунологов России</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.53529/2500-1175-2025-2-4-14</article-id><article-id custom-type="elpub" pub-id-type="custom">adair-201</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>ОБЗОР</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>REVIEW</subject></subj-group></article-categories><title-group><article-title>Генетические, морфологические и функциональные особенности триптазы человека</article-title><trans-title-group xml:lang="en"><trans-title>Genetic, morphological and functional characteristics of human tryptase</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-1387-7760</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Тарасова</surname><given-names>Н. Е.</given-names></name><name name-style="western" xml:lang="en"><surname>Tarasova</surname><given-names>N. E.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Тарасова Наталия Евгеньевна — к. м. н., доцент кафедры пропедевтики детских болезней</p><p>344022, г. Ростов-на-Дону, пер. Нахичеванский, 29</p></bio><bio xml:lang="en"><p>Natalia Evgenievna Tarasova — Cand. Sci., Associate Professor of Department of Propaedeutics Children’s Diseases</p><p>29 Nakhichevansky Lane, 344022, Rostov-on-Don</p></bio><email xlink:type="simple">nataly-alex@mail.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/0000-0003-4525-1500</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Лебеденко</surname><given-names>А. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Lebedenko</surname><given-names>A. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Лебеденко Александр Анатольевич — д. м. н., профессор, заведующий кафедрой детских болезней № 2 </p><p>344022, г. Ростов-на-Дону, пер. Нахичеванский, 29</p></bio><bio xml:lang="en"><p>Alexander Anatolyevich Lebedenko — Dr. Sci., Professor, head of Department of Children’s Diseases № 2</p><p>29 Nakhichevansky Lane, 344022, Rostov-on-Don</p></bio><email xlink:type="simple">leb.rost@rambler.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/0000-0002-3769-8014</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Семерник</surname><given-names>О. Е.</given-names></name><name name-style="western" xml:lang="en"><surname>Semernik</surname><given-names>O. E.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Семерник Ольга Евгеньевна — д. м. н., доцент кафедры детских болезней № 2 </p><p>344022, г. Ростов-на-Дону, пер. Нахичеванский, 29</p></bio><bio xml:lang="en"><p>Olga Evgenievna Semernik — Dr. Sci., Associate Professor of Department of Children’s Diseases № 2,</p><p>29 Nakhichevansky Lane, 344022, Rostov-on-Don</p></bio><email xlink:type="simple">semernick@mail.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/0000-0001-6113-9776</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Добаева</surname><given-names>Н. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Dobaeva</surname><given-names>N. M.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Добаева Наталья Михайловна — к. м. н., доцент, заведующая кафедрой общей и клинической биохимии № 2</p><p>344022, г. Ростов-на-Дону, пер. Нахичеванский, 29</p></bio><bio xml:lang="en"><p>Natalia Mikhailovna Dobaeva — Cand. Sci., Associate Professor, Head of the Department of General and Clinical Biochemistry № 2</p><p>29 Nakhichevansky Lane, 344022, Rostov-on-Don</p></bio><email xlink:type="simple">dobaeva_nm@rostgmu.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/0009-0009-1324-2635</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Скосарь</surname><given-names>В. О.</given-names></name><name name-style="western" xml:lang="en"><surname>Skosar</surname><given-names>V. O.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Скосарь Виктория Олеговна — врач клинико-лабораторной диагностики, ГБУРО «Центр по профилактике и борьбе со СПИД»</p><p>344022, г. Ростов-на-Дону, пер. Нахичеванский, 29</p><p> </p></bio><bio xml:lang="en"><p>Viktoria Olegovna Skosar — Clinical Laboratory Diagnostics Physician, State Budgetary Institution of the Rostov Region «Center for AIDS Prevention and Control»</p><p>29 Nakhichevansky Lane, 344022, Rostov-on-Don</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/0009-0000-8152-7151</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Хейгетян</surname><given-names>Н. Ю.</given-names></name><name name-style="western" xml:lang="en"><surname>Haygetyan</surname><given-names>N. Yu.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Хейгетян Нина Юрьевна — ассистент кафедры общей и клинической биохимии № 2 </p><p>344022, г. Ростов-на-Дону, пер. Нахичеванский, 29</p></bio><bio xml:lang="en"><p>Nina Yuryevna Haygetyan — Assistant of Department of General and Clinical Biochemistry No. 2</p><p>29 Nakhichevansky Lane, 344022, Rostov-on-Don</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/0000-0003-2286-3969</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Кривохлябов</surname><given-names>И. П.</given-names></name><name name-style="western" xml:lang="en"><surname>Krivokhlyabov</surname><given-names>I. P.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Кривохлябов Илья Петрович — к. м. н., доцент кафедры патологической физиологии</p><p>344022, г. Ростов-на-Дону, пер. Нахичеванский, 29</p></bio><bio xml:lang="en"><p>Ilya Petrovich Krivokhlyabov — Cand. Sci., Associate Professor, Department of Pathophysiology</p><p>29 Nakhichevansky Lane, 344022, Rostov-on-Don</p></bio><email xlink:type="simple">krivokhliabov_ip@rostgmu.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/0009-0001-6010-7526</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Шкильнюк</surname><given-names>С. П.</given-names></name><name name-style="western" xml:lang="en"><surname>Shkilnyuk</surname><given-names>S. P.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Шкильнюк Степан Петрович — студент педиатрического факультета </p><p>344022, г. Ростов-на-Дону, пер. Нахичеванский, 29</p></bio><bio xml:lang="en"><p>Stepan Petrovich Shkilnyuk — student of the Pediatric Faculty</p><p>29 Nakhichevansky Lane, 344022, Rostov-on-Don</p></bio><email xlink:type="simple">stepanshkilnuk@gmail.com</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Федеральное государственное бюджетное образовательное учреждение высшего образования «Ростовский государственный медицинский университет» Министерства здравоохранения Российской Федерации</institution></aff><aff xml:lang="en"><institution>Rostov state medical university</institution></aff></aff-alternatives><pub-date pub-type="collection"><year>2025</year></pub-date><pub-date pub-type="epub"><day>11</day><month>08</month><year>2025</year></pub-date><volume>0</volume><issue>2</issue><fpage>4</fpage><lpage>14</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Тарасова Н.Е., Лебеденко А.А., Семерник О.Е., Добаева Н.М., Скосарь В.О., Хейгетян Н.Ю., Кривохлябов И.П., Шкильнюк С.П., 2025</copyright-statement><copyright-year>2025</copyright-year><copyright-holder xml:lang="ru">Тарасова Н.Е., Лебеденко А.А., Семерник О.Е., Добаева Н.М., Скосарь В.О., Хейгетян Н.Ю., Кривохлябов И.П., Шкильнюк С.П.</copyright-holder><copyright-holder xml:lang="en">Tarasova N.E., Lebedenko A.A., Semernik O.E., Dobaeva N.M., Skosar V.O., Haygetyan N.Y., Krivokhlyabov I.P., Shkilnyuk S.P.</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://adair.elpub.ru/jour/article/view/201">https://adair.elpub.ru/jour/article/view/201</self-uri><abstract><sec><title>Актуальность</title><p>Актуальность. Триптаза, фермент тучных клеток, играет значимую роль в диагностике и патогенезе аллергических и воспалительных заболеваний. Базовый уровень триптазы в сыворотке используется как индикатор для диагностики заболеваний, сопровождающихся активацией тучных клеток, которые могут сопровождаться тяжелыми аллергическими реакциями и анафилаксией. Изучение изоформ триптазы, их структурных и функциональных вариаций, помогает понять генетическую предрасположенность и механизмы воспалительных заболеваний, таких как бронхиальная астма и хроническое воспаление.</p></sec><sec><title>Материалы и методы</title><p>Материалы и методы. Проведено детальное исследование структуры и функций различных изоформ триптазы. Изучены биохимические особенности различных изоформ триптазы человека. Анализ включал изучение генов TPSAB1, TPSB2, TPSG1 и TPSD1, кодирующих разные формы триптазы, а также оценку их активности. Изучена секреция триптазы, а также факторы, влияющие на ее секрецию.</p></sec><sec><title>Результаты</title><p>Результаты. Триптаза, фермент тучных клеток, представлена четырьмя основными изоформами — α, β, γ и δ. Среди них α- и β-триптазы являются секретируемыми, при этом β-триптаза обладает наибольшей каталитической активностью, тогда как α-триптаза имеет низкую ферментативную активность. Гетеротетрамеры α/β обладают уникальной активностью по сравнению с гомотетрамерами β, задействуя молекулы-мишени, неактивные для β-гомотетрамеров, что оказывает влияние на активность тучных клеток. Гены триптазы расположены на хромосоме 16 и имеют высокую степень гомологии. Важные гены TPSAB1 и TPSB2 кодируют активные формы, а увеличение числа копий TPSAB1 приводит к повышению базальных уровней триптазы, увеличивая риск развития аллергических реакций. Триптаза играет роль в воспалительных и аллергических процессах, в том числе в дегрануляции тучных клеток, влияя на сосудистую проницаемость и привлечение лейкоцитов. </p></sec><sec><title>Заключение</title><p>Заключение. Собранные данные о секреции и функциях триптазы, продуцируемой тучными клетками, позволяют считать ее многофункциональным медиатором, воздействующим посредством специфических молекулярных и клеточных механизмов. Триптаза играет важную роль в патогенезе воспалительных процессов и аллергических реакций в разнообразных органах и системах, включая респираторную систему и кожу. Понимание биохимических характеристик и генетических особенностей изоформ триптазы открывает возможности для разработки новых методов диагностики и лечения аллергических заболеваний с высокой социальной значимостью.</p></sec></abstract><trans-abstract xml:lang="en"><sec><title>Introduction</title><p>Introduction. Tryptase, a mast cell-derived protease, plays a significant role in the diagnosis and pathogenesis of allergic and inflammatory diseases. The baseline serum tryptase level is used as a biomarker for the diagnosis of conditions associated with mast cell activation, which may be accompanied by severe allergic reactions and anaphylaxis. Studying tryptase isoforms, along with their structural and functional variations, aids in understanding genetic predisposition and the mechanisms underlying inflammatory diseases such as bronchial asthma and chronic inflammation.</p></sec><sec><title>Materials and Methods</title><p>Materials and Methods. A detailed investigation of the structure and function of various tryptase isoforms was conducted. Biochemical properties human tryptase isoforms were examined. The analysis included the study of the genes TPSAB1, TPSB2, TPSG1, and TPSD1, which encode different forms of tryptase, and the assessment of their activity. Tryptase secretion has been investigated, along with various factors influencing its release.</p></sec><sec><title>Results</title><p>Results. Tryptase, a mast cell-derived enzyme, is represented by four major isoforms—α, β, γ, and δ. Among the secreted isoforms, α- and β-tryptases are the most prominent, β-tryptase exhibits the highest catalytic activity, whereas α-tryptase demonstrates limited enzymatic function. The tryptase genes are located on chromosome 16 and show a high degree of homology. Key genes TPSAB1 and TPSB2 encode active forms of tryptase, and an increased number of TPSAB1 copies leads to elevated baseline tryptase levels, heightening the risk of allergic reactions. Tryptase plays a role in inflammatory and allergic processes, including mast cell degranulation, affecting vascular permeability and leukocyte recruitment.</p></sec><sec><title>Conclusion</title><p>Conclusion. The collected data on the secretion and functions of tryptase produced by mast cells suggest that it can be regarded as a multifunctional mediator, acting through specific molecular and cellular mechanisms. Tryptase is critically involved in the pathogenesis of inflammatory processes and allergic responses across multiple organs and systems, including the respiratory tract and the skin. Understanding the biochemical characteristics and genetic features of tryptase isoforms opens new opportunities for the development of diagnostic and therapeutic approaches for high-impact allergic diseases.</p></sec></trans-abstract><kwd-group xml:lang="ru"><kwd>триптаза</kwd><kwd>тучные клетки</kwd><kwd>секреция</kwd><kwd>фермент</kwd><kwd>маркер</kwd></kwd-group><kwd-group xml:lang="en"><kwd>tryptase</kwd><kwd>mast cells</kwd><kwd>secretion</kwd><kwd>enzyme</kwd><kwd>marker</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">Elieh A., Komi D., Wöhrl S. et al. Mast Cell Biology at Molecular Level: a Comprehensive Review. Clin Rev Allergy Immunol. 2020; 58 (3): 342–365. https://doi.org/10.1007/s12016-019-08769-2.</mixed-citation><mixed-citation xml:lang="en">Elieh A. Komi D., Wöhrl S. et al. Mast Cell Biology at Molecular Level: a Comprehensive Review. 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