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Fibrinogen Vicenza and Genova II: two new cases of congenital dysfibrinogenemia with isolated defect of fibrin monomer polymerization and inhibitory activity on normal coagulation. 1987

F Rodeghiero, and G C Castaman, and A Dal Belin Peruffo, and E Dini, and A Galletti, and E Barone, and G Gastaldi
Department of Hematology, San Bortolo Hospital, Vicenza, Italy.

Two new cases of congenital dysfibrinogenemia are presented in which defective fibrin monomer polymerization and inhibitory activity on normal coagulation were observed. They have been tentatively called fibrinogen Vicenza and Genova II. The first was discovered in a family with mild bleeding diathesis, the second in an asymptomatic family. In almost all reported cases of fibrinogens with defective fibrin monomer polymerization, additional functional or structural defects have been detected. In our cases, on the contrary, detailed investigations failed to show any other abnormality. Fibrinogen Genova II is apparently identical to fibrinogen Baltimore IV, whereas fibrinogen Vicenza is similar to fibrinogen Troyes and Genova I, but also exerts an evident inhibitory activity on normal coagulation and differs from fibrinogen Genova II and Baltimore IV showing a different kinetic pattern of fibrin monomer polymerization.

UI MeSH Term Description Entries
D008297 Male Males
D004591 Electrophoresis, Polyacrylamide Gel Electrophoresis in which a polyacrylamide gel is used as the diffusion medium. Polyacrylamide Gel Electrophoresis,SDS-PAGE,Sodium Dodecyl Sulfate-PAGE,Gel Electrophoresis, Polyacrylamide,SDS PAGE,Sodium Dodecyl Sulfate PAGE,Sodium Dodecyl Sulfate-PAGEs
D005338 Fibrin Fibrinogen Degradation Products Soluble protein fragments formed by the proteolytic action of plasmin on fibrin or fibrinogen. FDP and their complexes profoundly impair the hemostatic process and are a major cause of hemorrhage in intravascular coagulation and fibrinolysis. Antithrombin VI,Fibrin Degradation Product,Fibrin Degradation Products,Fibrin Fibrinogen Split Products,Degradation Product, Fibrin,Degradation Products, Fibrin,Product, Fibrin Degradation
D005340 Fibrinogen Plasma glycoprotein clotted by thrombin, composed of a dimer of three non-identical pairs of polypeptide chains (alpha, beta, gamma) held together by disulfide bonds. Fibrinogen clotting is a sol-gel change involving complex molecular arrangements: whereas fibrinogen is cleaved by thrombin to form polypeptides A and B, the proteolytic action of other enzymes yields different fibrinogen degradation products. Coagulation Factor I,Factor I,Blood Coagulation Factor I,gamma-Fibrinogen,Factor I, Coagulation,gamma Fibrinogen
D005341 Fibrinolysin A product of the lysis of plasminogen (profibrinolysin) by PLASMINOGEN activators. It is composed of two polypeptide chains, light (B) and heavy (A), with a molecular weight of 75,000. It is the major proteolytic enzyme involved in blood clot retraction or the lysis of fibrin and quickly inactivated by antiplasmins. Plasmin,Fibrogammin,Glu-Plasmin,Protease F,Thrombolysin,Glu Plasmin
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D000347 Afibrinogenemia A deficiency or absence of FIBRINOGEN in the blood. Fibrinogen Deficiency,Afibrinogenemia, Congenital,Congenital Afibrinogenaemia,Congenital Afibrinogenemia,Deficiency, Fibrinogen,Familial Afibrinogenemia,Hypofibrinogenemia, Congenital,Afibrinogenaemia, Congenital,Afibrinogenaemias, Congenital,Afibrinogenemia, Familial,Afibrinogenemias,Afibrinogenemias, Congenital,Afibrinogenemias, Familial,Congenital Afibrinogenaemias,Congenital Afibrinogenemias,Congenital Hypofibrinogenemia,Congenital Hypofibrinogenemias,Familial Afibrinogenemias,Fibrinogen Deficiencies,Hypofibrinogenemias, Congenital
D012967 Sodium Dodecyl Sulfate An anionic surfactant, usually a mixture of sodium alkyl sulfates, mainly the lauryl; lowers surface tension of aqueous solutions; used as fat emulsifier, wetting agent, detergent in cosmetics, pharmaceuticals and toothpastes; also as research tool in protein biochemistry. Sodium Lauryl Sulfate,Irium,Dodecyl Sulfate, Sodium,Lauryl Sulfate, Sodium,Sulfate, Sodium Dodecyl,Sulfate, Sodium Lauryl
D013918 Thrombin Time Clotting time of PLASMA mixed with a THROMBIN solution. It is a measure of the conversion of FIBRINOGEN to FIBRIN, which is prolonged by AFIBRINOGENEMIA, abnormal fibrinogen, or the presence of inhibitory substances, e.g., fibrin-fibrinogen degradation products, or HEPARIN. BATROXOBIN, a thrombin-like enzyme unaffected by the presence of heparin, may be used in place of thrombin. Reptilase Time,Reptilase Times,Thrombin Times,Time, Reptilase,Time, Thrombin,Times, Reptilase,Times, Thrombin

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