title: Measurement of factor XIII activity in plasma.
abstract: Coagulation factor XIII (FXIII) is converted by thrombin and Ca(2+) into an active transglutaminase (FXIIIa) in the final phase of coagulation cascade. Its main function is the mechanical stabilization of fibrin clot and its protection from fibrinolysis by cross-linking of fibrin chains and α(2)-plasmin inhibitor to fibrin. In non-substituted patients FXIII deficiency is a severe hemorrhagic diathesis, not infrequently with fatal consequences. The main reason for using FXIII assays is the diagnosis of FXIII deficiency. The aim of this review is to provide a comprehensive critical evaluation of the methods reported for the determination of FXIII activity in the plasma. Such methods are based on two principles: 1) measurement of labeled amines incorporated by FXIIIa into a glutamine residue of a substrate protein, 2) monitoring ammonia released from a peptide bound glutamine residue by FXIIIa using NAD(P)H dependent glutamate dehydrogenase indicator reaction. The incorporation assays are sensitive, but cumbersome and time-consuming, they are difficult to standardize and cannot be automated. The ammonia release assays are less sensitive, but quick, well standardized, and can be automated; this type of assay is recommended for the screening of FXIII deficiency. The traditional clot solubility assay should not be used for this purpose.
authors: Clinical Research Center, University of Debrecen, Medical and Health Science Center, Debrecen, Hungary.
authors: Éva
authors: É
authors: Katona
authors: Krisztina
authors: K
authors: Pénzes
authors: Éva
authors: É
authors: Molnár
authors: László
authors: L
authors: Muszbek
mesh_terms: D005176:Factor XIII / Q000032:analysis / Q000378:metabolism*; D005177:Factor XIII Deficiency / Q000097:blood*; D006801:Humans; D013917:Thrombin / Q000378:metabolism; D013927:Thrombosis / Q000097:blood
chemical_list: D005176:Factor XIII; D013917:Thrombin