A Specific, Fluorescent Activity Staining Procedure Applied to Plasma and Red Blood Cells in Congenital Factor XIII Deficiency

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Abstract
The activity staining procedure introduced by Stenberg and Stenflo was applied to studies on human blood transamidases (transglutaminases, endo-.gamma.-glutamine:.epsilon.-lysine transferases, e.g., factor [F] XIII). The technique combined agarose gel electrophoresis with activity staining based on the transamidase catalyzed incorporation of monodansylthiacadaverine (N-(5-amino-3-thiapentyl)-5-dimethylamino-1-naphtalenesulfonamide) into casein. The method permits detection of plasma FXIII activity down to 1% of the normal adult standard. The technique was used on plasma from 2 patients with tentative congenital plasma FXIII deficiency (based on clot solubility). No activity was found in platelet-poor and in platelet-rich plasma which confirmed the diagnosis. In the erythrocytes studied in 1 of the 2 patients transamidase activity was present, which means separate genetic determinations of the plasma and red blood cell transamidases. Using immunoelectrophoresis, the plasma FXIII b subunit was 43 and 44% of the concentration in normal standard plasma.