title: Factor XIII: a coagulation factor with multiple plasmatic and cellular functions.
abstract: 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.
authors: 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. muszbek@med.unideb.hu
authors: László
authors: L
authors: Muszbek
authors: Zsuzsanna
authors: Z
authors: Bereczky
authors: Zsuzsa
authors: Z
authors: Bagoly
authors: István
authors: I
authors: Komáromi
authors: Éva
authors: É
authors: Katona
mesh_terms: D000818:Animals; D001773:Blood Cells / Q000502:physiology; D001777:Blood Coagulation / Q000502:physiology; D001846:Bone Development / Q000502:physiology; D002199:Capillary Permeability / Q000502:physiology; D002316:Cardiotonic Agents / Q000378:metabolism; D002356:Cartilage / Q000254:growth & development; D002468:Cell Physiological Phenomena; D015536:Down-Regulation; D005176:Factor XIII / Q000737:chemistry / Q000378:metabolism*; D026122:Factor XIIIa / Q000378:metabolism; D005260:Female; D005337:Fibrin / Q000378:metabolism; D006801:Humans; D018919:Neovascularization, Physiologic; D010949:Plasma / Q000502:physiology*; D011247:Pregnancy / Q000502:physiology; D014945:Wound Healing / Q000502:physiology
chemical_list: D002316:Cardiotonic Agents; D005337:Fibrin; D005176:Factor XIII; D026122:Factor XIIIa