bloodjourГематология и трансфузиологияRussian journal of hematology and transfusiology0234-57302411-3042ООО Издательский дом «Практика»10.35754/0234-5730-2022-67-4-570-578bloodjour-411Research ArticleОБЗОРЫREVIEWSФизиологическая и патологическая роль фактора свертывания крови XIIPhysiological and pathological role of factor XIIhttps://orcid.org/0000-0002-6991-7437ЯковлеваЕ. В.YakovlevaE. V.

Яковлева Елена Владимировна, кандидат медицинских наук, гематолог, научный сотрудник клинико-диагностического отделения гематологии и нарушений гемостаза

125167, Москва

Elena V. Yakovleva, Cand. Sci. (Med.), Hematologist, Researcher, Clinical Diagnostic Department of Hematology and Hemostasis Disorders

125167, Moscow

hemophilia2012@mail.ruhttps://orcid.org/0000-0001-7074-0926ЗозуляН. И.ZozulyaN. I.

Зозуля Надежда Ивановна, доктор медицинских наук, заведующая клинико-диагностическим отделением гематологии и нарушений гемостаза

125167, Москва

Nadezhda I. Zozulya, Dr. Sci. (Med.), Head of the Clinical Diagnostic Department of Hematology and Hemostasis Disorders

125167, Moscow

zozulya.n@blood.ruФГБУ «Национальный медицинский исследовательский центр гематологии» Министерства здравоохранения Российской ФедерацииРоссияNational Medical Research Center for HematologyRussian Federation202227122022674570578Copyright © Яковлева Е.В., Зозуля Н.И., 20222022Яковлева Е.В., Зозуля Н.И.Yakovleva E.V., Zozulya N.I.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.htjournal.ru/jour/article/view/411Введение

Введение. Наиболее распространенным представлением о функции фактора свертывания крови XII (FXII) является его участие во внутреннем пути свертывания крови. Однако биологическая роль FXII многообразна.

Цель – обзор разнообразных биологических функций FXII.

Основные сведения

Основные сведения. FXII является сериновой протеазой. Структура FXII имеет высокую степень гомологии с плазминогеном, тканевым активатором плазминогена и урокиназным активатором плазминогена. Активированный FXII (FXIIa) имеет пять субстратов: высокомолекулярный кининоген, прекалликреин, FXI, плазминоген, белки комплемента (C1s, С1r). FXII обеспечивает гемостатическое равновесие, участвуя в процессах свертывания крови и фибринолиза. FXII регулирует воспалительные и аллергические реакции, взаимодействуя с калликреин-кининовой системой и системой комплемента. FXII имеет биологическую активность в различных клетках in vivo: эндотелиоцитах, тромбоцитах, моноцитах, нейтрофилах, фибробластах, дендритных клетках, что определяет его многообразную роль в физиологических и патологических процессах.

Introduction

Introduction. The most widely accepted notion of the function of blood clotting factor XII (FXII, Hageman factor) is its involvement in the internal blood clotting pathway. However, the biological role of FXII is diverse.

Aim – to review the diverse biological functions of FXII.

Main findings

Main findings. FXII is a serine protease. The structure of FXII has a high degree of homology with plasminogen, tissue plasminogen activator and urokinase plasminogen activator. Activated FXII (FXIIa) has five substrates: high-molecular kininogen, precallikrein, FXI, plasminogen, complement proteins (C1s, C1r). FXII provides hemostatic balance by participating in the processes of blood clotting and fibrinolysis. FXII regulates inflammatory and allergic reactions by interacting with the kallikreinkinin system and the complement system. FXII has biological activity in various cells in vivo: endotheliocytes, platelets, monocytes, neutrophils, fi broblasts, dendritic cells, which determines its diverse role in physiological and pathological processes.

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The authors declare that there are no conflicts of interest present.