Factor XIII Active Protein | F13A1 active protein
Factor XIII, Human
EC 2.3.2.13
Affinity Purified
EC 2.3.2.13
Affinity Purified; F13A1 active protein
A2: Mr 75,000
B2: Mr 88,000
A2: Mr 75,000
B2: Mr 88,000
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).
NCBI and Uniprot Product Information
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: 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
Research Articles on F13A1
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Product Notes
The F13A1 f13a1 (Catalog #AAA634628) is an Active Protein produced from Human plasma and is intended for research purposes only. The product is available for immediate purchase. It is sometimes possible for the material contained within the vial of "Factor XIII, Active Protein" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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