Catalytic activity:Protein glutamine + alkylamine = protein N (5) -alkylglutamine + NH (3) . ,cofactor:Binds 1 calcium ion per subunit. ,Disease:Defects in F13A1 are the cause of F13A deficiency [MIM:134570]. F13A deficiency is an autosomal recessive disorder characterized by a life-long bleeding tendency , impaired wound healing and spontaneous abortion in affected women. In addition to the common presentation such as subcutaneous and intramuscular haematomas , severe bleeding such as intracranial hemorrhages may occur. ,Function: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. ,online information:Factor XIII entry ,online information:The Singapore human mutation and polymorphism database ,polymorphism:There are four main allelic forms of this protein; F13A*1A , F13A*1B , F13A*2A and F13A*2B. In addition two other intermediate forms (F13A* (2) A and F13A* (2) B) seem to exist. The sequence shown is that of F13A* (2) B. ,PTM:The activation peptide is released by thrombin. ,similarity:Belongs to the transglutaminase superfamily. Transglutaminase family. ,subcellular location:Secreted into the blood plasma. Cytoplasmic in most tissues , but also secreted in the blood plasma. ,subunit:Tetramer of two A chains and two B chains. ,
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