coagulation factor XIII B chain
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A F13B gene provides instructions for making one part, the B subunit, of a protein called factor XIII. This protein is part of a group of related proteins called coagulation factors that are essential for normal blood clotting. They work together as part of the coagulation cascade, which is a series of chemical reactions that forms blood clots in response to injury. After an injury, clots seal off blood vessels to stop bleeding and trigger blood vessel repair. Factor XIII acts at the end of the cascade to strengthen and stabilize newly formed clots, preventing further blood loss.
Normal function
A F13B gene provides instructions for making one part, the B subunit, of a protein called factor XIII. This protein is part of a group of related proteins called coagulation factors that are essential for normal blood clotting. They work together as part of the coagulation cascade, which is a series of chemical reactions that forms blood clots in response to injury. After an injury, clots seal off blood vessels to stop bleeding and trigger blood vessel repair. Factor XIII acts at the end of the cascade to strengthen and stabilize newly formed clots, preventing further blood loss.
Factor XIII in the bloodstream is made of two A subunits (produced from the F13A1 gene) and two B subunits (produced from the F13B gene). The role of the B subunits is to carry and stabilize the A subunits, protecting them from being broken down. When a new blood clot forms, the A and B subunits separate from one another, and the A subunits are cut (cleaved) to produce the active form of factor XIII (factor XIIIa). The active protein links together molecules of fibrin, the material that forms the clot, which strengthens the clot and keeps other molecules from breaking it down.
Studies suggest that factor XIII has additional functions, although these are less well understood than its role in blood clotting. Specifically, factor XIII is likely involved in other aspects of wound healing, immune system function, maintaining pregnancy, bone formation, and the growth of new blood vessels (angiogenesis).
Conditions referring to this gene or chromosome
Other names
coagulation factor XIII B chain precursor
coagulation factor XIII, B polypeptide
fibrin-stabilizing factor B subunit
FXIIIB
protein-glutamine gamma-glutamyltransferase B chain
TGase
transglutaminase B chain
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Leitura adicional e referências
- coagulation factor XIII B chain; MedlinePlus, National Library of Medicine.
- Bagoly Z, Koncz Z, Harsfalvi J, Muszbek L. Factor XIII, clot structure, thrombosis. Thromb Res. 2012 Mar;129(3):382-7. doi: 10.1016/j.thromres.2011.11.040. Epub 2011 Dec 24.
- Biswas A, Ivaskevicius V, Seitz R, Thomas A, Oldenburg J. An update of the mutation profile of Factor 13 A and B genes. Blood Rev. 2011 Sep;25(5):193-204. doi: 10.1016/j.blre.2011.03.001. Epub 2011 Jun 2.
- Biswas A, Ivaskevicius V, Thomas A, Oldenburg J. Coagulation factor XIII deficiency. Diagnosis, prevalence and management of inherited and acquired forms. Hamostaseologie. 2014;34(2):160-6. doi: 10.5482/HAMO-13-08-0046. Epub 2014 Feb 7.
- Hethershaw EL, Cilia La Corte AL, Duval C, Ali M, Grant PJ, Ariens RA, Philippou H. The effect of blood coagulation factor XIII on fibrin clot structure and fibrinolysis. J Thromb Haemost. 2014 Feb;12(2):197-205. doi: 10.1111/jth.12455.
- Levy JH, Greenberg C. Biology of Factor XIII and clinical manifestations of Factor XIII deficiency. Transfusion. 2013 May;53(5):1120-31. doi: 10.1111/j.1537-2995.2012.03865.x. Epub 2012 Aug 28.
- Muszbek L, Bereczky Z, Bagoly Z, Komaromi I, Katona E. Factor XIII: a coagulation factor with multiple plasmatic and cellular functions. Physiol Rev. 2011 Jul;91(3):931-72. doi: 10.1152/physrev.00016.2010.
- Schroeder V, Kohler HP. Factor XIII deficiency: an update. Semin Thromb Hemost. 2013 Sep;39(6):632-41. doi: 10.1055/s-0033-1353392. Epub 2013 Aug 8.
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