Coagulation factor XIII A chain is a protein that in humans is encoded by the F13A1gene.
This gene encodes the coagulation factor XIII A subunit. Coagulation factor XIII is the last zymogen to become activated in the blood coagulation cascade. Plasma factor XIII is a heterotetramer composed of 2 A subunits and 2 B subunits. The A subunits have catalytic function, and the B subunits do not have enzymatic activity and may serve as plasma carrier molecules. Platelet factor XIII is composed of just 2 A subunits, which are identical to those of plasma origin. Upon cleavage of the activation peptide by thrombin and in the presence of calcium ion, the plasma factor XIII dissociates its B subunits and yields the same active enzyme, factor XIIIa, as platelet factor XIII. This enzyme acts as a transglutaminase to catalyze the formation of gamma-glutamyl-epsilon-lysine crosslinking between fibrin molecules, thus stabilizing the fibrin clot. It also crosslinks alpha-2-plasmin inhibitor, or fibronectin, to the alpha chains of fibrin. Factor XIII deficiency is classified into two categories: type I deficiency, characterized by the lack of both the A and B subunits; and type II deficiency, characterized by the lack of the A subunit alone. These defects can result in a lifelong bleeding tendency, defective wound healing, and habitual abortion.
^Carrell, N A; Erickson H P, McDonagh J (Jan 1989). "Electron microscopy and hydrodynamic properties of factor XIII subunits". J. Biol. Chem. (UNITED STATES) 264 (1): 551–6. ISSN0021-9258. PMID2491853.Cite uses deprecated parameters (help)
^Achyuthan, K E; Rowland T C, Birckbichler P J, Lee K N, Bishop P D, Achyuthan A M (Sep 1996). "Hierarchies in the binding of human factor XIII, factor XIIIa, and endothelial cell transglutaminase to human plasma fibrinogen, fibrin, and fibronectin". Mol. Cell. Biochem. (NETHERLANDS) 162 (1): 43–9. ISSN0300-8177. PMID8905624.Cite uses deprecated parameters (help)
Bishop PD, Teller DC, Smith RA, et al. (1990). "Expression, purification, and characterization of human factor XIII in Saccharomyces cerevisiae.". Biochemistry29 (7): 1861–9. doi:10.1021/bi00459a028. PMID2184890.
Board PG, Webb GC, McKee J, Ichinose A (1988). "Localization of the coagulation factor XIII A subunit gene (F13A) to chromosome bands 6p24----p25.". Cytogenet. Cell Genet.48 (1): 25–7. doi:10.1159/000132580. PMID2903011.
Ichinose A, Hendrickson LE, Fujikawa K, Davie EW (1987). "Amino acid sequence of the a subunit of human factor XIII.". Biochemistry25 (22): 6900–6. doi:10.1021/bi00370a025. PMID3026437.
Takagi T, Doolittle RF (1974). "Amino acid sequence studies on factor XIII and the peptide released during its activation by thrombin.". Biochemistry13 (4): 750–6. doi:10.1021/bi00701a018. PMID4811064.
Yee VC, Pedersen LC, Bishop PD, et al. (1995). "Structural evidence that the activation peptide is not released upon thrombin cleavage of factor XIII.". Thromb. Res.78 (5): 389–97. doi:10.1016/0049-3848(95)00072-Y. PMID7660355.
Coggan M, Baker R, Miloszewski K, et al. (1995). "Mutations causing coagulation factor XIII subunit A deficiency: characterization of the mutant proteins after expression in yeast.". Blood85 (9): 2455–60. PMID7727776.
Kaczmarek E, Liu Y, Berse B, et al. (1995). "Biosynthesis of plasma factor XIII: evidence for transcription and translation in hepatoma cells.". Biochim. Biophys. Acta1247 (1): 127–34. PMID7873582.
Suzuki K, Iwata M, Ito S, et al. (1994). "Molecular basis for subtypic differences of the "a" subunit of coagulation factor XIII with description of the genesis of the subtypes.". Hum. Genet.94 (2): 129–35. PMID7913909.