Fibrinogen Aarhus--a new case of dysfibrinogenemia.
Fibrinogen Aarhus was found in a woman with slightly prolonged whole blood clotting time. The thrombin induced clotting of plasma and purified fibrinogen was much prolonged. Kinetic analysis of FPA and FPB release revealed larger apparent Km and Vmax values for fibrinogen Aarhus than for normal fibrinogen. No clot formation of fibrinogen Aarhus was demonstrated in the presence of Batroxobin and the release of FPA was slower than normal. Upon addition of the clotting enzyme from Agkistrodon contortrix contortrix clotting did occur but the clotting time was much prolonged in comparison with normal fibrinogen. The turbidity of fibrin gels obtained from fibrinogen Aarhus was similar to normal fibrin gels at low thrombin concentrations. Increasing thrombin concentration resulted in appearance of degradation products in the fibrin gels from fibrinogen Aarhus and at the same time a relative increase in turbidity of the gels was observed. Possibly reasons for the slow release of fibrinopeptides, the delayed gelation, and susceptibility to degradation by thrombin are discussed.
D000328:Adult; D000596:Amino Acids / Q000032:analysis; D012103:Batroxobin / Q000494:pharmacology; D001778:Blood Coagulation Disorders / Q000097:blood* / Q000151:congenital; D001780:Blood Coagulation Tests; D003435:Crotalid Venoms / Q000494:pharmacology; D004591:Electrophoresis, Polyacrylamide Gel; D005260:Female; D005340:Fibrinogen / Q000032:analysis*; D015241:Fibrinogens, Abnormal; D005341:Fibrinolysin; D005344:Fibrinopeptide A / Q000032:analysis; D005345:Fibrinopeptide B / Q000032:analysis; D005782:Gels; D006801:Humans; D007700:Kinetics; D009391:Nephelometry and Turbidimetry; D011863:Radioimmunoassay; D013917:Thrombin / Q000494:pharmacology
C031442:Agkistrodon venoms; D000596:Amino Acids; D003435:Crotalid Venoms; D015241:Fibrinogens, Abnormal; D005782:Gels; C048788:fibrinogen Aarhus; D005344:Fibrinopeptide A; D005345:Fibrinopeptide B; D005340:Fibrinogen; D012103:Batroxobin; D013917:Thrombin; D005341:Fibrinolysin