Biochemistry
| Synonym | Fibrin stabilizing factor |
|---|---|
| Molecular mass | 320.000 Da |
| Synthesis | Liver, megakaryocytes |
| Half life | 8 days |
| Plasma concentration | 2 mg/l |
| Normal range | 69 - 143% or 0.69 - 1.43 U/ml |
Factor XIII is the proenzyme (zymogen) of a transglutaminase, which is activated by thrombin. The active factor XIIIa covalently crosslinks the α and β chains thus stabilizing the fibrin network, which develops after the thrombin-mediated cleavage of fibrinogen. The thrombus is thus protected against a premature lysis. At the same time, factor XIIIa mediates the binding of the plasmin inhibitor (α
2
-antiplasmin) and fibronectin to fibrin. Factor XIII is found in plasma, in thrombocytes and certain tissues (e.g. placenta). In plasma factor XIII is present as a tetramer (A2B2), in which the active site is localized within the A chain.
Clinical significance
Hereditary factor XIII deficiency is extremely rare. An acquired deficiency is observed as a consequence of hepatocyte dysfunction and during asparaginase therapy. In addition, consumption and loss coagulopathy, sepsis, leukemia and acute venous thromboembolism can induce a factor XIII deficiency.
Indication
- Impaired wound healing
- Suspected acquired factor XIII deficiency (consumption and loss coagulopathy, postoperative bleeding, liver disease)
- Suspected hereditary factor XIII deficiency (impaired wound healing with abnormal scar formation, cerebral and soft tissue bleeding and bleeding into joints)
Literature
- Muszbek L et al. Blood Coagulation Factor XIII: Structure and Function. Thromb Res 94, 271-305, 1999.
- Kohler et al. A Common Coding Polymorphism in the FXIII A-subunit Gene (FXIIIVAL34LEU) Affects Cross-linking Activity. Thromb Haemost 80, 704, 1998.
- Anwar R et al. Genotype/Phenotype Correlations for Coagulation Factor XIII: Specific Normal Polymorphisms Are Associated With High or Low Factor XIII Specific Activity. Blood 93, 897-905, 1999.
- Dempfle CE et al. Microtiter assay for measurement of factor XIII activity in plasma. J Lab Clin Med 119, 522-528, 1992.
- Parameswaran KN, Cheng XF, Chen EC, Velasco PT, Wilson JH, Lorand L.; Hydrolysis of gamma:epsilon isopeptides by cytosolic transglutaminases and by coagulation factor XIIIa, J Biol Chem 10311-10317, 1997.
- Biswas A, Ivaskevicius V, Thomas A, Oldenburg J. Coagulation factor XIII deficiency. Diagnosis, prevalence and management of inherited and acquired forms. Hämostaseologie 34(2), 160-6, 2014.
- Dickneite G, Herwald H, Korte W, Allanore Y, Denton CP, Matucci Cerinic M. Coagulation factor XIII: a multifunctional transglutaminase with clinical potential in a range of conditions. Thromb Haemost. Apr 113(4), 686-97, 2015.