功能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.
疾病相关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.
序列相似性Belongs to the transglutaminase superfamily. Transglutaminase family.
翻译后修饰The activation peptide is released by thrombin.
细胞定位Cytoplasm. Secreted. Secreted into the blood plasma. Cytoplasmic in most tissues, but also secreted in the blood plasma.