Factor XIII

Title: Factor XIII
CAS Registry Number: 9013-56-3
CAS Name: Blood-coagulation factor XIII
Additional Names: fibrin-stabilizing factor; FSF; fibrinase; Laki-Lorand factor; LLF
Trademarks: Fibrogammin (Centeon)
Literature References: Plasma enzyme precursor which, when activated by thrombin in the presence of Ca2+, converts soluble fibrin, q.v. gel to a tough insoluble clot: Laki, Lorand, Science 108, 280 (1948); Lorand, Nature 166, 694 (1950). During the terminal stage of blood-clotting, factor XIII catalyzes the formation of cross-links in the fibrin gel by eliminating NH3 in the transamidation reaction in which the e-amino group of lysine from a fibrin molecule forms a peptide linkage with a glutaminyl residue from a nearby fibrin monomer: Lorand et al., Biochem. Biophys. Res. Commun. 25, 629 (1966); Fuller, Doolittle, ibid. 694. Trypsin, papain, and reptilase also activate factor XIII: Lorand et al., ibid. 31, 222 (1968). Structure contains two each of two types of soluble proteinaceous subunits called the a and b chains; mol wt of subunits each about 80,000: Schwartz et al., J. Biol. Chem. 248, 1395 (1973). Amino acid sequence studies: Holbrook et al., Biochem. J. 135, 901 (1973). Clinical studies: Cucuianu et al., Thromb. Diath. Haemorrh. 30, 480 (1973). Review: L. Lorand, Ann. N.Y. Acad. Sci. 202, 6-30 (1972); A. G. Loewy, ibid. 41-58; C. G. Curtis, L. Lorand, Haemostasis: Biochemistry, Physiology & Pathology, D. Ogston, B. Bennett, Eds. (Wiley-Interscience, New York, 1977) pp 186-201; eidem, Methods Enzymol. 45B, 177-191 (1976).
Properties: uv max: 280 nm (E1% 13.8). Retains activity in plasma kept at 0°; inactivated in a few days at room temperature. Activity destroyed if serum heated at 60° for 10 min. Does not dialyse out from plasma. Inactivated by Echis coloratus venom. Pretreatment with compounds containing an SH-group, such as cysteine, reduced glutathione, DL-penicillamine, or mercapto-1-propanol prevents inactivation as well as restores activity after venom treatment.
Absorption maximum: uv max: 280 nm (E1% 13.8)
Therap-Cat: Antihemorrhagic.
Keywords: Hemostatic.
Fadrozole Fagarine Famoxadone Famphur Fantofarone

Factor XIII crosslinks fibrin
coagulation factor XIII,
A1 polypeptide
Identifiers
Symbol F13A1
Alt. symbols F13A
Entrez 2162
HUGO 3531
OMIM 134570
RefSeq NM_000129
UniProt P00488
Other data
Locus Chr. 6 p24.2-p23
coagulation factor XIII,
B polypeptide
Identifiers
Symbol F13B
Entrez 2165
HUGO 3534
OMIM 134580
RefSeq NM_001994
UniProt P05160
Other data
Locus Chr. 1 q31-q32.1

Factor XIII or fibrin stabilizing factor is an enzyme (EC 2.3.2.13) of the blood coagulation system that crosslinks fibrin.