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.
Involvement in disease
Defects in F13A1 are the cause of factor XIII subunit A deficiency (FA13AD) [MIM:613225]. 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.
The activation peptide is released by thrombin.
Cytoplasm. Secreted. Secreted into the blood plasma. Cytoplasmic in most tissues, but also secreted in the blood plasma.