Factor XIIIa ist ein Gen, das durch das Symbol F13A1 kodiert wird. Andere Namen sind: Coagulation factor XIII A chain; Coagulation Protein-glutamine gamma-glutamyltransferase A chain; Transglutaminase A chain; F13A1; F13A. Factor XIIIa hat eine Masse von 83.27kDa, eine Aminosäurelänge von 732, und ist an Factor XIII subunit A deficiency beteiligt.
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Gen- und Proteininformationen
UniProt Zusammenfassung
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.
Entrez Zusammenfassung
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.
Rolle bei Krankheiten
Factor XIII subunit A deficiency: An autosomal recessive hematologic disorder characterized by a life-long bleeding tendency, impaired wound healing and spontaneous abortion in affected women.
Sequenzähnlichkeiten
Belongs to the transglutaminase superfamily. Transglutaminase family.
Posttranslationale Modifikation
The activation peptide is released by thrombin.
Zellort
Cytoplasm. Secreted.
Secreted into the blood plasma. Cytoplasmic in most tissues, but also secreted in the blood plasma.
