Factor XIIIa Protein | F13A1 protein

Factor XIIIa, Human (Fibrinoligase, Plasma Transglutaminase, Fibrin Stabilizing Factor)

Cat. Num. AAA634650

• Gene Names: F13A1; F13A
• Purity: Affinity Purified; Plasma Factor XIIIa is generated by cleavage of homogenous plasma Factor XIIIa by alpha-thrombin, in the presence of EDTA. The A2'B2 subunit is purified by ion and affinity chromatography.
• Synonyms: Factor XIIIa; Human (Fibrinoligase; Plasma Transglutaminase; Fibrin Stabilizing Factor); F13A1 protein

• Host: Human plasma
• Purity/Purification: Affinity Purified; Plasma Factor XIIIa is generated by cleavage of homogenous plasma Factor XIIIa by alpha-thrombin, in the presence of EDTA. The A2'B2 subunit is purified by ion and affinity chromatography.
• Form/Format: Supplied in 50% glycerol, 0.5mM EDTA.

• Extinction Coefficient: E1%1cm, 280nm=13.8 (Inferred from factor XIII)
• Isoelectric Point: 5.2 (Inferred from factor XIII)
• Mode of Action: Plasma transglutaminase
• Structure: Tetramer (A2'B2) in the absence of calcium, two identical A chains (Mr=71,000) each containing 6 free sulfhydryls and an active site, two identical B subunit (Mr=88,000) identical subunits, A (Mr=75,000), B (Mr=88,000)
• Preparation and Storage: -20 degree C

Related Product Information for F13A1 protein

Factor XIIIa (FXIIIa) (fibrinoligase, plasma transglutaminase, fibrin stabilizing factor, E.C. 2.3.2.13) is a glutaminyl-peptide g-glutamyl transferase functioning in the final stages of the coagulation cascade, stabilizing the fibrin clot by crosslinking the a- and g-chains of fibrin to form homopolymers (1-4). Several unique features of FXIIIa set it apart from proteins with which it is generally associated. Factor XIIIa is the only non-proteolytic enzyme of the coagulation cascade. Unlike other transamidases, it exists totally in zymogenic form and is the only sulfhydrylase enzyme which functions extracellularly.

Plasma FXIIIa is a tetramer, (Mr=312,000), composed of 2 pairs of non-identical subunits (A2B2) (4-7). Platelet FXIII is a dimer (Mr=46,000), composed of only a pair of identical A subunits (A2) as is factor XIII from all intracellular sources (7-11). The A subunit contains 6 free sulfhydryl groups, one of which is the active center (12).
The conversion of plasma FXIII (A2B2) to the active transglutaminase, FXIIIa (A2') results from the thrombin catalyzed hydrolysis of the Arg36-Gly37 peptide bond at the NH2-terminal of the A subunit (13). The conformational change induced by this cleavage results in exposure of the active site cysteine. Full activity is achieved only after the Ca2+ dependent (Kd=10e-3M) dissociation of the B subunit dimer from the A2' dimer (14-16). Dissociation of the A2'B2 complex is enhanced by binding to the region of fibrin(ogen) containing residues 242-424 of the Aa chain (14-16). Thus, at normal plasma concentrations of FXIII (90nM), fibrinogen (8uM) and Ca2+ (2.5mM free), all the FXIIIa generated would be in the fully activated (A2') molecular form. Other physiologically relevant crosslinked complexes generated by FXIIIa include fibrin-fibrinogen (17), fibrin-fibronectin (18), fibrin-vWF (19), fibrin-a2antiplasmin (20), fibrin-actin (21), fibrin-thrombospondin, fibronectin-collagen (19), vWF-collagen (19), actin-myosin (21,22), factor V-factor V (23). In addition to clot stabilization, FXIIIa is thought to function in various processes involving cell proliferation, such as wound healing, tissue remodeling, atherosclerosis, and tumor growth.

Product Categories/Family for F13A1 protein

Molecular Biology; MB-Coagulation Factors

NCBI and Uniprot Product Information

• NCBI GI #: 412153
• NCBI GeneID: 2162
• UniProt Accession #: P00488; Q59HA7; Q8N6X2; Q96P24; Q9BX29
• Molecular Weight: 312,000
• NCBI Official Full Name: factor XIIIa
• NCBI Official Synonym Full Names: coagulation factor XIII, A1 polypeptide
• NCBI Official Symbol: F13A1
• NCBI Official Synonym Symbols: F13A
• NCBI Protein Information: 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)
• UniProt Protein Name: Coagulation factor XIII A chain
• Protein Family: Coagulation factor
• UniProt Gene Name: F13A1
• UniProt Synonym Gene Names: F13A; Coagulation factor XIIIa
• UniProt Entry Name: F13A_HUMAN

NCBI Description

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]

Uniprot Description

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: Transferase; EC 2.3.2.13

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