Factor XIIIa Recombinant Rabbit Monoclonal Antibody - BSA and Azide free [PSH21-18] (HA751787)

UNIPROT #

P00488

PROTEIN NAME

Factor XIIIa

SYNONYMS

bA525O21.1 (coagulation factor XIII, A1 polypeptide) antibody;Coagulation factor XIII A chain antibody;Coagulation factor XIII A1 polypeptide antibody;Coagulation factor XIII A1 subunit antibody;Coagulation factor XIII, A polypeptide antibody;Coagulation factor XIIIa antibody;F13A antibody;F13A_HUMAN antibody;F13a1 antibody;Factor XIIIA antibody;Fibrin stabilizing factor, A subunit antibody;Fibrinoligase antibody;FSF, A subunit antibody;Protein glutamine gamma glutamyltransferase A chain antibody;Protein-glutamine gamma-glutamyltransferase A chain antibody;TGase antibody;Transglutaminase A chain antibody;Transglutaminase, plasma antibody;Transglutaminase. plasma antibody

SUBCELLULAR LOCATION

Secreted, Cytoplasm.

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 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.Factor XIIIa has been identified in platelets, megakaryocytes, and fibroblast-like mesenchymal or histiocytic cells in the placenta, uterus, and prostate, monocytes and macrophages and dermal dendritic cells. Anti- Factor XIIIa has been found to be useful in differentiating between dermatofibroma (almost all cases +), dermatofibrosarcoma protuberans (-/+) and desmoplastic malignant melanoma (-). Anti-factor XIIIa positivity is also seen in capillary hemagioblastoma, hemangioendothelioma, hemangiopericytoma, xanthogranuloma, xanthoma, hepatocellular carcinoma, glomus tumor, and meningioma.