Literature DB >> 2939591

Fibrinogen Aarhus--a new case of dysfibrinogenemia.

B Hessel, S Stenbjerg, J Dyr, B Kudryk, L Therkildsen, B Blombäck.   

Abstract

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.

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Year:  1986        PMID: 2939591     DOI: 10.1016/0049-3848(86)90193-3

Source DB:  PubMed          Journal:  Thromb Res        ISSN: 0049-3848            Impact factor:   3.944


  3 in total

1.  Aberrant hepatic processing causes removal of activation peptide and primary polymerisation site from fibrinogen Canterbury (A alpha 20 Val --> Asp).

Authors:  S O Brennan; B Hammonds; P M George
Journal:  J Clin Invest       Date:  1995-12       Impact factor: 14.808

Review 2.  Structure and function of human fibrinogen inferred from dysfibrinogens.

Authors:  Michio Matsuda; Teruko Sugo
Journal:  Int J Hematol       Date:  2002-08       Impact factor: 2.490

3.  Fibrinogen αC-regions are not directly involved in fibrin polymerization as evidenced by a "Double-Detroit" recombinant fibrinogen mutant and knobs-mimic peptides.

Authors:  Cédric Duval; Aldo Profumo; Anna Aprile; Annalisa Salis; Enrico Millo; Gianluca Damonte; Julia S Gauer; Robert A S Ariëns; Mattia Rocco
Journal:  J Thromb Haemost       Date:  2020-01-29       Impact factor: 5.824

  3 in total

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