Factor XIII: a coagulation factor with multiple plasmatic and cellular functions

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, m...

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Bibliographic Details
Published in:Physiological reviews 2011-07, Vol.91 (3), p.931-972
Main Authors: Muszbek, László, Bereczky, Zsuzsanna, Bagoly, Zsuzsa, Komáromi, István, Katona, Éva
Format: Article
Language:English
Subjects:
Animals
Blood Cells - physiology
Blood clots
Blood Coagulation - physiology
Blood platelets
Bone Development - physiology
Capillary Permeability - physiology
Cardiotonic Agents - metabolism
Cartilage - growth & development
Cell Physiological Phenomena
Coagulation
Cytoplasm
Down-Regulation
Factor XIII - chemistry
Factor XIII - metabolism
Factor XIIIa - metabolism
Female
Fibrin - metabolism
Humans
Neovascularization, Physiologic
Physiology
Plasma - physiology
Pregnancy - physiology
Proteins
Wound Healing - physiology
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Summary: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.
ISSN:0031-9333
1522-1210
DOI:10.1152/physrev.00016.2010