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F13A1

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.

Involvement in disease

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.

Post-translational modifications

The activation peptide is released by thrombin.

Sequence Similarities

Belongs to the transglutaminase superfamily. Transglutaminase family.

Cellular localization

Alternative names

F13A, F13A1, Coagulation factor XIII A chain, Coagulation factor XIIIa, Protein-glutamine gamma-glutamyltransferase A chain, Transglutaminase A chain

swissprot:P00488 entrezGene:2162 omim:134570