Fibrin - Stabilizing Factor
نویسندگان
چکیده
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 that rendered clots prepared from purified fibrinogen insoluble in weak acid and urea was reported by Robbins’ in 1944. Laki and Lorand2 confirmed this observation in 1948 and designated this factor as the “fibrin-stabilizing factor.” In 1963, the International Committee on New Blood Clotting Factors accepted this entity as a distinct coagulation factor and gave it the designation factor XIII. Patients with factor XIII deficiency have a tendency to bleed and show poor wound healing. In these patients, the usual tests for blood coagulation give normal results except for a considerable degree of crumbling of the clot and a striking diminution of the amplitude of the thromboelastogram.3 The diagnosis is confirmed by the rapid dissolution of the clot obtained by recalcification of the plasma in a 5 M urea or a 1% monochioroacetic acid solution. The factor XIII deficiency has been documented in at least 44 individuals from 27 families,3 including a single case from Pakistan reported by Zahir4 from this laboratory. In this communication, four additional cases of bleeding disorders associated with the fibrin-stabilizing factor (factor XIII) deficiency from two families will be reported.
منابع مشابه
The effect of fibrin-stabilizing factor on the subunit structure of human fibrin.
The formation of human fibrin from fibrinogen has been examined by polyacrylamide gel electrophoresis in sodium dodecyl sulfate, a method which separates a mixture of proteins on the basis of differences in molecular weight. It has been found that the plasma from a patient with a congenital deficiency of fibrin-stabilizing factor forms clots lacking the cross links among the alpha- and gammacha...
متن کاملIsolation and properties of a thrombin-sensitive protein from human blood platelets.
A thrombin-sensitive protein has been isolated from human blood platelets by gel electrophoresis followed by filtration through Sephadex. The isolated protein was homogeneous on disc gel and behaved like a P-globulin. In immunoelectrophoresis, a single precipitin line was observed with antiplatelet serum or anti-fibrin-stabilizing factor serum. The precipitin line as well as the protein band on...
متن کاملCross-Linking of a2-Plasmin Inhibitor to Fibrin by Fibrin-stabilizing Factor
itor in blood plasma is higher than that in serum obtained from the blood clotted in the presence ofcalcium ions, but is the same as that in serum obtained in the absence of calcium ions. Radiolabeled a2-plasmin inhibitor was covalently bound to fibrin only when calcium ions were present at the time of clotting of plasma or fibrinogen. Whereas, when batroxobin, a snake venom enzyme that lacks t...
متن کاملCurrent understanding in diagnosis and management of factor XIII deficiency
Factor XIII or "fibrin-stabilizing factor," is a transglutaminase circulates in the blood circulation as a hetero tetramer with two catalytic A subunits and two carrier B subunits. This important coagulation factor has a crucial role in clotting cascade and produces strong covalent bonds between soluble formed fibrin monomers during coagulation. This stable cross linked fibrin strands are resis...
متن کاملFibrinolytic and fibrin stabilizing activity of synovial membranes.
Most tissues exhibit fibrinolytic activity because of their content of plasminogen activator (Albrechtsen, 1958; Astrup and Stage, 1952; Pandolfi, Nilsson, Robertson, and Isacson, 1967; Sayers, Tyler, and Lack, 1965). This activity might be demonstrated by applying the tissue itself or a tissue extract on a fibrin plate (Astrup and Permin, 1947; Caughey and Highton, 1967) or by the histiochemic...
متن کامل