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.
Factor XIII subunit A deficiency
FA13AD
An autosomal recessive hematologic disorder characterized by a life-long bleeding tendency, impaired wound healing and spontaneous abortion in affected women.
None
The disease is caused by variants affecting the gene represented in this entry.
The activation peptide is released by thrombin.
Belongs to the transglutaminase superfamily. Transglutaminase family.
Proteins
Immuno-oncology
83267Da
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