FunctionFactor 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 diseaseDefects 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.
Sequence similaritiesBelongs to the transglutaminase superfamily. Transglutaminase family.
Post-translational modificationsThe activation peptide is released by thrombin.
Cellular localizationCytoplasm. Secreted. Secreted into the blood plasma. Cytoplasmic in most tissues, but also secreted in the blood plasma.