protein

Factor XIII, Human. EC 2.3.2.13. Affinity Purified

MBS634628

0.1 mg

505 USD

Protein

Factor XIII, Human. EC 2.3.2.13. Affinity Purified

[Factor XIII]

[factor XIII, partial; Coagulation factor XIII A chain; coagulation factor XIII A chain; TGase; factor XIIIa; fibrinoligase; FSF, A subunit; coagulation factor XIIIa; transglutaminase A chain; transglutaminase. plasma; fibrin stabilizing factor, A subunit; coagulation factor XIII, A polypeptide; protein-glutamine gamma-glutamyltransferase A chain; bA525O21.1 (coagulation factor XIII, A1 polypeptide); coagulation factor XIII, A1 polypeptide; Protein-glutamine gamma-glutamyltransferase A chain; Transglutaminase A chain]

[F13A1; F13A1; F13A; F13A; Coagulation factor XIIIa]

F13A_HUMAN

Human plasma

Supplied in 0.5mM EDTA, 50% glycerol/H2O.

4.8mg/ml

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 6 months after receipt at -20 degree C. FOr maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

Specific Activity: 151 U/mg. 1unit is equal to the Factor XIII activity in 1ml normal plasma. Isoelectric Point: 5.2. Structure: 2 pairs of identical subunits, A2B2. A2: Mr 75,000. B2: Mr 88,000

Specific Activity: 151 U/mg. 1unit is equal to the Factor XIII activity in 1ml normal plasma. Isoelectric Point: 5.2. Structure: 2 pairs of identical subunits, A2B2. A2: Mr 75,000. B2: Mr 88,000. Carbohydrate Content: A Chain: 1%; B Chain: 5%. Extinction Coefficient (1%, 1cm, 280nm): 13.8

Molecular Biology; MB-Coagulation Factors

Factor XIII is the zymogenic form of the glutaminyl-peptide g-glutamyl transferase factor XIIIa (fibrinoligase, plasma transglutaminase, fibrin stabilizing factor, E.C. 2.3.2.13) (1-3). Factor XIII is unique among transamidases in that it is a zymogen in vivo (2). Factor XIII is found both extracellularly in plasma and intracellularly in platelets, megakaryocytes, monocytes, placenta, uterus, liver and prostrate tissues. Plasma factor XIII is synthesized in the liver and circulates as a tetramer (Mr 20,000), composed of 2 pairs of nonidentical subunits (A2B2) (4). The intracellular forms are synthesized in the tissues where they reside as dimers (Mr 6000) of 2 identical A chains (A2) (7-11). The A subunits of plasma and intracellular forms of factor XIII are functionally identical. The A subunit contains 6 free sulfhydryl groups one of which is the active site (12). The concentration of factor XIII in plasma (A2B2) is approximately 30mg/ml (8). It is the last of the zymogens to become activated in the coagulation cascade and it is the only enzyme in this system that is not a serine protease. The conversion of plasma factor XIII (A2B2) to the active transamidase factor XIIIa (A2') results from hydrolysis of the Arg36-Gly37 at the NH2-terminus of the A subunit by thrombin (13). Full expression of activity is achieved only after the Ca2+ (Kd-3M) and fibrin(ogen) (Kd-8M) dependent dissociation of the B subunit dimer from the A2' dimer (14-16).

182837

AAA52489.1

P00488

320kD

Common Pathway (106060); Complement And Coagulation Cascades Pathway (83073); Complement And Coagulation Cascades Pathway (484); Formation Of Fibrin Clot (Clotting Cascade) Pathway (106057); Hemostasis Pathway (106028); Platelet Activation, Signaling And Aggregation Pathway (106034); Platelet Degranulation Pathway (106050); Response To Elevated Platelet Cytosolic Ca2+ Pathway (106048)

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 comprised only of 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. [provided by RefSeq, Jul 2008]

F13A1: Factor XIII is activated by thrombin and calcium ion to a transglutaminase that catalyzes the formation of gamma-glutamyl- epsilon-lysine cross-links between fibrin chains, thus stabilizing the fibrin clot. Also cross-link alpha-2-plasmin inhibitor, or fibronectin, to the alpha chains of fibrin. Defects in F13A1 are the cause of factor XIII subunit A deficiency (FA13AD). FA13AD is an autosomal recessive disorder characterized by a life-long bleeding tendency, impaired wound healing and spontaneous abortion in affected women. Belongs to the transglutaminase superfamily. Transglutaminase family. Protein type: EC 2.3.2.13; Transferase. Chromosomal Location of Human Ortholog: 6p25.3-p24.3. Cellular Component: extracellular region. Molecular Function: protein-glutamine gamma-glutamyltransferase activity; metal ion binding. Biological Process: platelet activation; platelet degranulation; peptide cross-linking; blood coagulation. Disease: Thrombophilia Due To Thrombin Defect; Myocardial Infarction, Susceptibility To; Factor Xiii, A Subunit, Deficiency Of

0.1 mg

505 USD