Four patients with congenital defiother family were found to have varyciency of fibrin-stabilizing factor (facing degrees of factor XIII deficiency. tor XIII) from two families have been The observations support the hypothedescribed. The mother and the sibs in sis of autosomal recessive inheritance one family and both parents in the of factor XIII deficiency. T HE EXISTENCE OF A BLOOD FACTOR th...

Factor XIII deficiency is a congenital defect of the blood coagulation system. Activated factor XIII is the fibrin cross-linking enzyme that catalyzes the formation of N 6 y-glutamyllysyl bonds in fibrin. Congenital deficiency of factor XIII has been described in approximately 65 separate families. It is apparent that this is a hereditary trait, but the mode of inheritance is unclear. In this s...

Coagulaon factor XIII gene, protein structure and funcon Coagulaon factor XIII (FXIII) is a tetrameric (FXIII- A2B2) pro-transglutaminase enzyme with an essenal role in the final stage of coagulaon cascade by cross linking the fibrin monomers and stabilizing the fibrin clot. Congenital FXIII deficiency is a rare bleeding disorder, with an autosomal recessive trait inheritance, and a fre...

We have previously demonstrated that increasing factor XIII concentrations above that present in plasma (1 U/mL) results in the formation of very high molecular weight alpha fate polyacrylamide and agarose gel electrophoresis (SDS-PAGE). In this report, we have examined the effect of such crosslinking on plasmic susceptibility of fibrin prepared from purified fibrinogen and from plasma in the p...

Four patients with congenital defiother family were found to have varyciency of fibrin-stabilizing factor (facing degrees of factor XIII deficiency. tor XIII) from two families have been The observations support the hypothedescribed. The mother and the sibs in sis of autosomal recessive inheritance one family and both parents in the of factor XIII deficiency. T HE EXISTENCE OF A BLOOD FACTOR th...

Factor XIII deficiency is a rare inherited coagulopathy. Factor XIII is the last clotting factor in the coagulation cascade to insure strength and stability to fibrin clots. Without this enzyme, the fibrous clot is unstable and nonresistant to fibrinolysis. Gravid women with this congenital disease are especially at risk for complications including miscarriages and hemorrhage without appropriat...

Homozygous patients with factor XIII deficiency are devoid of immunologically identifiable A protein, the active enzymatic component. Quantitative studies of transamidase activity of the factor are available in only a few cases, and the fibrin cross-linking pattern is not well known. The present paper deals with the quantitative estimation of factor XIII transamidase activity (dansylcadaverine ...

Human fibrin-stabilizing factor (Factor XIII) has been studied immunologically by the preparation of specific anti-Factor XIII antiserum in rabbits. On immunodiffusion it was found that normal plasma produced two precipitin lines. One of the precipitin lines was identical with that present in soluble platelet extract (the alpha-component), the other with that present in normal serum (beta-compo...

We have previously demonstrated that increasing factor XIII concentrations above that present in plasma (1 U/mI) results in the formation of very high molecular weight a fate polyacrylamide and agarose gel electrophoresis (SDSPAGE). In this report. we have examined the effect of such crosslinking on plasmic susceptibility of fibrin prepared from purified fibrinogen and from plasma in the presen...