Clinical Research Center and Thrombosis, Haemostasis and Vascular Biology Research Group of the Hungarian Academy of Sciences, University of Debrecen, Medical and Health Science Center, Debrecen, Hungary.
Physiol Rev. 2011 Jul;91(3):931-72. doi: 10.1152/physrev.00016.2010.
Factor XIII (FXIII) is unique among clotting factors for a number of reasons: 1) it is a protransglutaminase, which becomes activated in the last stage of coagulation; 2) it works on an insoluble substrate; 3) its potentially active subunit is also present in the cytoplasm of platelets, monocytes, monocyte-derived macrophages, dendritic cells, chondrocytes, osteoblasts, and osteocytes; and 4) in addition to its contribution to hemostasis, it has multiple extra- and intracellular functions. This review gives a general overview on the structure and activation of FXIII as well as on the biochemical function and downregulation of activated FXIII with emphasis on new developments in the last decade. New aspects of the traditional functions of FXIII, stabilization of fibrin clot, and protection of fibrin against fibrinolysis are summarized. The role of FXIII in maintaining pregnancy, its contribution to the wound healing process, and its proangiogenic function are reviewed in details. Special attention is given to new, less explored, but promising fields of FXIII research that include inhibition of vascular permeability, cardioprotection, and its role in cartilage and bone development. FXIII is also considered as an intracellular enzyme; a separate section is devoted to its intracellular activation, intracellular action, and involvement in platelet, monocyte/macrophage, and dendritic cell functions.
凝血因子 XIII（FXIII）因其诸多原因而在凝血因子中独具特色：1）它是一种前转谷氨酰胺酶，在凝血的最后阶段被激活；2）它作用于不溶性底物；3）其潜在的活性亚基也存在于血小板、单核细胞、单核细胞衍生的巨噬细胞、树突状细胞、软骨细胞、成骨细胞和成骨细胞的细胞质中；4）除了对止血的贡献外，它还具有多种细胞外和细胞内功能。本文综述了 FXIII 的结构和激活以及激活 FXIII 的生化功能及其下调，重点介绍了过去十年的新进展。总结了 FXIII 的传统功能（稳定纤维蛋白凝块和保护纤维蛋白免于纤维蛋白溶解）的新方面。详细综述了 FXIII 在维持妊娠、促进伤口愈合过程和促进血管生成功能中的作用。特别关注 FXIII 研究中较新的、探索较少但有前途的领域，包括抑制血管通透性、心脏保护以及在软骨和骨骼发育中的作用。FXIII 也被认为是一种细胞内酶；专门有一节介绍其细胞内激活、细胞内作用以及在血小板、单核细胞/巨噬细胞和树突状细胞功能中的作用。
