Coagulation factor XIII, A1 polypeptide

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External IDs	GeneCards: [1]
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Species	Human	Mouse
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Coagulation factor XIII A chain is a protein that in humans is encoded by the F13A1 gene.

Function

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.

Interactions

Coagulation factor XIII, A1 polypeptide has been shown to interact with F13B.

Further reading

• Adány R (1997). "Intracellular factor XIII: cellular distribution of factor XIII subunit a in humans". Semin. Thromb. Hemost. 22 (5): 399–408. doi:10.1055/s-2007-999038. PMID 8989823.
• Adány R, Bárdos H (2003). "Factor XIII subunit A as an intracellular transglutaminase". Cell. Mol. Life Sci. 60 (6): 1049–60. doi:10.1007/s00018-003-2178-9. PMID 12861374.
• Bereczky Z, Katona E, Muszbek L (2005). "Fibrin stabilization (factor XIII), fibrin structure and thrombosis". Pathophysiol. Haemost. Thromb. 33 (5–6): 430–7. doi:10.1159/000083841. PMID 15692256.
• Board P, Coggan M, Miloszewski K (1992). "Identification of a point mutation in factor XIII A subunit deficiency". Blood. 80 (4): 937–41. PMID 1353995.
• Kamura T, Okamura T, Murakawa M, Tsuda H, Teshima T, Shibuya T, Harada M, Niho Y (1992). "Deficiency of coagulation factor XIII A subunit caused by the dinucleotide deletion at the 5' end of exon III". J. Clin. Invest. 90 (2): 315–9. doi:10.1172/JCI115864. PMC 443104. PMID 1644910.
• Harsfalvi J, Fesus L, Tarcsa E, Laczko J, Loewy AG (1991). "The presence of a covalently cross-linked matrix in human platelets". Biochim. Biophys. Acta. 1073 (2): 268–74. doi:10.1016/0304-4165(91)90131-Y. PMID 2009280.
• Bishop PD, Teller DC, Smith RA, Lasser GW, Gilbert T, Seale RL (1990). "Expression, purification, and characterization of human factor XIII in Saccharomyces cerevisiae". Biochemistry. 29 (7): 1861–9. doi:10.1021/bi00459a028. PMID 2184890.
• Hilgenfeld R, Liesum A, Storm R, Metzner HJ, Karges HE (1990). "Crystallization of blood coagulation factor XIII by an automated procedure". FEBS Lett. 265 (1–2): 110–2. doi:10.1016/0014-5793(90)80896-Q. PMID 2365049.
• Carrell NA, Erickson HP, McDonagh J (1989). "Electron microscopy and hydrodynamic properties of factor XIII subunits". J. Biol. Chem. 264 (1): 551–6. PMID 2491853.
• Takahashi N, Takahashi Y, Putnam FW (1986). "Primary structure of blood coagulation factor XIIIa (fibrinoligase, transglutaminase) from human placenta". Proc. Natl. Acad. Sci. U.S.A. 83 (21): 8019–23. doi:10.1073/pnas.83.21.8019. PMC 386858. PMID 2877456.
• Grundmann U, Amann E, Zettlmeissl G, Küpper HA (1986). "Characterization of cDNA coding for human factor XIIIa". Proc. Natl. Acad. Sci. U.S.A. 83 (21): 8024–8. doi:10.1073/pnas.83.21.8024. PMC 386859. PMID 2877457.
• Ichinose A, Davie EW (1988). "Characterization of the gene for the a subunit of human factor XIII (plasma transglutaminase), a blood coagulation factor". Proc. Natl. Acad. Sci. U.S.A. 85 (16): 5829–33. doi:10.1073/pnas.85.16.5829. PMC 281858. PMID 2901091.
• 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. PMID 2903011.
• Ichinose A, Hendrickson LE, Fujikawa K, Davie EW (1987). "Amino acid sequence of the a subunit of human factor XIII". Biochemistry. 25 (22): 6900–6. doi:10.1021/bi00370a025. PMID 3026437.
• Takagi T, Doolittle RF (1974). "Amino acid sequence studies on factor XIII and the peptide released during its activation by thrombin". Biochemistry. 13 (4): 750–6. doi:10.1021/bi00701a018. PMID 4811064.
• Yee VC, Pedersen LC, Bishop PD, Stenkamp RE, Teller DC (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. PMID 7660355.
• Coggan M, Baker R, Miloszewski K, Woodfield G, Board P (1995). "Mutations causing coagulation factor XIII subunit A deficiency: characterization of the mutant proteins after expression in yeast". Blood. 85 (9): 2455–60. PMID 7727776.
• Kaczmarek E, Liu Y, Berse B, Chen CS, McDonagh J (1995). "Biosynthesis of plasma factor XIII: evidence for transcription and translation in hepatoma cells". Biochim. Biophys. Acta. 1247 (1): 127–34. doi:10.1016/0167-4838(94)00167-f. PMID 7873582.
• Yee VC, Pedersen LC, Le Trong I, Bishop PD, Stenkamp RE, Teller DC (1994). "Three-dimensional structure of a transglutaminase: human blood coagulation factor XIII". Proc. Natl. Acad. Sci. U.S.A. 91 (15): 7296–300. doi:10.1073/pnas.91.15.7296. PMC 44386. PMID 7913750.
• Suzuki K, Iwata M, Ito S, Matsui K, Uchida A, Mizoi Y (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. doi:10.1007/bf00202857. PMID 7913909.