Factor XIII Assay, Plasma

Plasma factor XIII (FXIII) is a tetrameric molecule composed of 2 α- and 2 β-subunits held together non covalently in a heterologous tetramer. Fibrin is the main protein constituent of the blood clot, which is stabilised by factor XIIIa through an amide or iso-peptide bond that ligates adjacent fibrin monomers. The α-subunit of FXIII contains the active site of the enzyme and is synthesised by hepatocytes, monocytes, and megakaryocytes. Thrombin cleavage of the α-subunits is necessary to activate plasma FXIII. The generation of FXIIIa in plasma can be triggered when as little as 1% to 2% of fibrinogen is converted to fibrin polymers. This indicates that FXIIIa begins stabilising fibrin polymers before a visible thrombus appears. Without FXIII, the fibrin strands are fragile and break away.

FXIII deficiency is a rare disease that causes a bleeding disorder (haemophilia). Most cases of FXIII deficiency are associated with alterations in the gene that encodes the catalytic A subunit of this transglutaminase that crosslinks the α and β chains of fibrin monomers to yield stable clots.

2 ml Citrate Plasma Frozen

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