| 释义 |
- Function
- Interactions
- References
- Further reading
{{Infobox_gene}}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.[1] Interactions Coagulation factor XIII, A1 polypeptide has been shown to interact with F13B.[2][3] References 1. ^{{cite web | title = Entrez Gene: F13A1 coagulation factor XIII, A1 polypeptide| url = https://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=2162| accessdate = }}
2. ^{{cite journal | vauthors = Carrell NA, Erickson HP, McDonagh J | title = Electron microscopy and hydrodynamic properties of factor XIII subunits | journal = J. Biol. Chem. | volume = 264 | issue = 1 | pages = 551–6 | date = Jan 1989 | pmid = 2491853 }}
3. ^{{cite journal | vauthors = Achyuthan KE, Rowland TC, Birckbichler PJ, Lee KN, Bishop PD, Achyuthan AM | title = Hierarchies in the binding of human factor XIII, factor XIIIa, and endothelial cell transglutaminase to human plasma fibrinogen, fibrin, and fibronectin | journal = Mol. Cell. Biochem. | volume = 162 | issue = 1 | pages = 43–9 | date = Sep 1996 | pmid = 8905624 | doi = 10.1007/bf00250994 }}
Further reading {{refbegin | 2}}- {{cite journal | vauthors = Adány R | title = Intracellular factor XIII: cellular distribution of factor XIII subunit a in humans. | journal = Semin. Thromb. Hemost. | volume = 22 | issue = 5 | pages = 399–408 | year = 1997 | pmid = 8989823 | doi = 10.1055/s-2007-999038 }}
- {{cite journal | vauthors = Adány R, Bárdos H | title = Factor XIII subunit A as an intracellular transglutaminase. | journal = Cell. Mol. Life Sci. | volume = 60 | issue = 6 | pages = 1049–60 | year = 2003 | pmid = 12861374 | doi = 10.1007/s00018-003-2178-9 }}
- {{cite journal | vauthors = Bereczky Z, Katona E, Muszbek L | title = Fibrin stabilization (factor XIII), fibrin structure and thrombosis. | journal = Pathophysiol. Haemost. Thromb. | volume = 33 | issue = 5-6 | pages = 430–7 | year = 2005 | pmid = 15692256 | doi = 10.1159/000083841 }}
- {{cite journal | vauthors = Board P, Coggan M, Miloszewski K | title = Identification of a point mutation in factor XIII A subunit deficiency. | journal = Blood | volume = 80 | issue = 4 | pages = 937–41 | year = 1992 | pmid = 1353995 | doi = }}
- {{cite journal | vauthors = Kamura T, Okamura T, Murakawa M, Tsuda H, Teshima T, Shibuya T, Harada M, Niho Y | title = Deficiency of coagulation factor XIII A subunit caused by the dinucleotide deletion at the 5' end of exon III. | journal = J. Clin. Invest. | volume = 90 | issue = 2 | pages = 315–9 | year = 1992 | pmid = 1644910 | pmc = 443104 | doi = 10.1172/JCI115864 }}
- {{cite journal | vauthors = Harsfalvi J, Fesus L, Tarcsa E, Laczko J, Loewy AG | title = The presence of a covalently cross-linked matrix in human platelets. | journal = Biochim. Biophys. Acta | volume = 1073 | issue = 2 | pages = 268–74 | year = 1991 | pmid = 2009280 | doi = 10.1016/0304-4165(91)90131-Y }}
- {{cite journal | vauthors = Bishop PD, Teller DC, Smith RA, Lasser GW, Gilbert T, Seale RL | title = Expression, purification, and characterization of human factor XIII in Saccharomyces cerevisiae. | journal = Biochemistry | volume = 29 | issue = 7 | pages = 1861–9 | year = 1990 | pmid = 2184890 | doi = 10.1021/bi00459a028 }}
- {{cite journal | vauthors = Hilgenfeld R, Liesum A, Storm R, Metzner HJ, Karges HE | title = Crystallization of blood coagulation factor XIII by an automated procedure. | journal = FEBS Lett. | volume = 265 | issue = 1-2 | pages = 110–2 | year = 1990 | pmid = 2365049 | doi = 10.1016/0014-5793(90)80896-Q }}
- {{cite journal | vauthors = Carrell NA, Erickson HP, McDonagh J | title = Electron microscopy and hydrodynamic properties of factor XIII subunits. | journal = J. Biol. Chem. | volume = 264 | issue = 1 | pages = 551–6 | year = 1989 | pmid = 2491853 | doi = }}
- {{cite journal | vauthors = Takahashi N, Takahashi Y, Putnam FW | title = Primary structure of blood coagulation factor XIIIa (fibrinoligase, transglutaminase) from human placenta. | journal = Proc. Natl. Acad. Sci. U.S.A. | volume = 83 | issue = 21 | pages = 8019–23 | year = 1986 | pmid = 2877456 | pmc = 386858 | doi = 10.1073/pnas.83.21.8019 }}
- {{cite journal | vauthors = Grundmann U, Amann E, Zettlmeissl G, Küpper HA | title = Characterization of cDNA coding for human factor XIIIa. | journal = Proc. Natl. Acad. Sci. U.S.A. | volume = 83 | issue = 21 | pages = 8024–8 | year = 1986 | pmid = 2877457 | pmc = 386859 | doi = 10.1073/pnas.83.21.8024 }}
- {{cite journal | vauthors = Ichinose A, Davie EW | title = Characterization of the gene for the a subunit of human factor XIII (plasma transglutaminase), a blood coagulation factor. | journal = Proc. Natl. Acad. Sci. U.S.A. | volume = 85 | issue = 16 | pages = 5829–33 | year = 1988 | pmid = 2901091 | pmc = 281858 | doi = 10.1073/pnas.85.16.5829 }}
- {{cite journal | vauthors = Board PG, Webb GC, McKee J, Ichinose A | title = Localization of the coagulation factor XIII A subunit gene (F13A) to chromosome bands 6p24----p25. | journal = Cytogenet. Cell Genet. | volume = 48 | issue = 1 | pages = 25–7 | year = 1988 | pmid = 2903011 | doi = 10.1159/000132580 }}
- {{cite journal | vauthors = Ichinose A, Hendrickson LE, Fujikawa K, Davie EW | title = Amino acid sequence of the a subunit of human factor XIII. | journal = Biochemistry | volume = 25 | issue = 22 | pages = 6900–6 | year = 1987 | pmid = 3026437 | doi = 10.1021/bi00370a025 }}
- {{cite journal | vauthors = Takagi T, Doolittle RF | title = Amino acid sequence studies on factor XIII and the peptide released during its activation by thrombin. | journal = Biochemistry | volume = 13 | issue = 4 | pages = 750–6 | year = 1974 | pmid = 4811064 | doi = 10.1021/bi00701a018 }}
- {{cite journal | vauthors = Yee VC, Pedersen LC, Bishop PD, Stenkamp RE, Teller DC | title = Structural evidence that the activation peptide is not released upon thrombin cleavage of factor XIII. | journal = Thromb. Res. | volume = 78 | issue = 5 | pages = 389–97 | year = 1995 | pmid = 7660355 | doi = 10.1016/0049-3848(95)00072-Y }}
- {{cite journal | vauthors = Coggan M, Baker R, Miloszewski K, Woodfield G, Board P | title = Mutations causing coagulation factor XIII subunit A deficiency: characterization of the mutant proteins after expression in yeast. | journal = Blood | volume = 85 | issue = 9 | pages = 2455–60 | year = 1995 | pmid = 7727776 | doi = }}
- {{cite journal | vauthors = Kaczmarek E, Liu Y, Berse B, Chen CS, McDonagh J | title = Biosynthesis of plasma factor XIII: evidence for transcription and translation in hepatoma cells. | journal = Biochim. Biophys. Acta | volume = 1247 | issue = 1 | pages = 127–34 | year = 1995 | pmid = 7873582 | doi = 10.1016/0167-4838(94)00167-f }}
- {{cite journal | vauthors = Yee VC, Pedersen LC, Le Trong I, Bishop PD, Stenkamp RE, Teller DC | title = Three-dimensional structure of a transglutaminase: human blood coagulation factor XIII. | journal = Proc. Natl. Acad. Sci. U.S.A. | volume = 91 | issue = 15 | pages = 7296–300 | year = 1994 | pmid = 7913750 | pmc = 44386 | doi = 10.1073/pnas.91.15.7296 }}
- {{cite journal | vauthors = Suzuki K, Iwata M, Ito S, Matsui K, Uchida A, Mizoi Y | title = Molecular basis for subtypic differences of the "a" subunit of coagulation factor XIII with description of the genesis of the subtypes. | journal = Hum. Genet. | volume = 94 | issue = 2 | pages = 129–35 | year = 1994 | pmid = 7913909 | doi = 10.1007/bf00202857 }}
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