Fibrinogen Milano II : A congenital dysfibrinogenaemia associated with juvenile arterial and venous thrombosis
article
A congenitally abnormal fibrinogen was isolated from blood of a young man with deep-vein thrombosis. Two other affected members of this family had three episodes of severe arterial thrombosis. The fibrinogen showed a delayed clotting by thrombin, but a normal clotting by Arvin??, Reptilase??, and prothrombin-staphylocoagulase complex. Analysis of the fibrinopeptides A and B by high performance liquid chromatography did not reveal an abnormal peptide structure. The rate of release of A and B peptides by thrombin was strongly delayed, whereas the rate of release of fibrinopeptide A by Arvin?? appeared to be normal. The fibrin polymerization rate was normal. Interactions between the abnormal fibrinogen, platelets and the fibrinolytic interaction between fibrinogen Milano II and thrombin is associated with a defective binding of thrombin to the fibrin moiety of the abnormal fibrinogen. Chemicals/CAS: fibrinogen, 9001-32-5; Fibrin, 9001-31-4; fibrinogen Milano II; Fibrinogen, 9001-32-5; Fibrinopeptide A, 25422-31-5; Fibrinopeptide B, 36204-23-6; Thrombin, EC 3.4.21.5
Topics
fibrinogenartery thrombosisblood and hemopoietic systemcardiovascular systemcongenital disorderdeep vein thrombosisdysfibrinogenemiahigh performance liquid chromatographyhumanperipheral vascular systempriority journalthrombosisAdultBlood Coagulation DisordersCase ReportFibrinFibrinogenFibrinopeptide AFibrinopeptide BHumanMalePedigreePlatelet AggregationSupport, Non-U.S. Gov'tThrombinThrombophlebitisThrombosis
TNO Identifier
230034
ISSN
03406245
Source
Thrombosis and Haemostasis, 55(1), pp. 131-135.
Pages
131-135
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