Literature DB >> 3705000

Fibrinogen Milano II: a congenital dysfibrinogenaemia associated with juvenile arterial and venous thrombosis.

F Haverkate, J Koopman, C Kluft, A D'Angelo, M Cattaneo, P M Mannucci.   

Abstract

A congenitally abnormal fibrinogen was isolated from blood of a young man with deep-vein thrombosis. Two other affected members of his 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 system were also normal. Evidence is presented that the defective interaction between fibrinogen Milano II and thrombin is associated with a defective binding of thrombin to the fibrin moiety of the abnormal fibrinogen.

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Year:  1986        PMID： 3705000

Source DB:  PubMed          Journal:  Thromb Haemost        ISSN： 0340-6245            Impact factor:   5.249


  6 in total

1.  Crystal structure of the complex between thrombin and the central "E" region of fibrin.

Authors:  Igor Pechik; Joel Madrazo; Michael W Mosesson; Irene Hernandez; Gary L Gilliland; Leonid Medved
Journal:  Proc Natl Acad Sci U S A       Date:  2004-02-20       Impact factor: 11.205

2.  Crystal structure of fibrinogen-Aalpha peptide 1-23 (F8Y) bound to bovine thrombin explains why the mutation of Phe-8 to tyrosine strongly inhibits normal cleavage at Arg-16.

Authors:  M G Malkowski; P D Martin; S T Lord; B F Edwards
Journal:  Biochem J       Date:  1997-09-15       Impact factor: 3.857

3.  Fibrinogen Lima: a homozygous dysfibrinogen with an A alpha-arginine-141 to serine substitution associated with extra N-glycosylation at A alpha-asparagine-139. Impaired fibrin gel formation but normal fibrin-facilitated plasminogen activation catalyzed by tissue-type plasminogen activator.

Authors:  H Maekawa; K Yamazumi; S Muramatsu; M Kaneko; H Hirata; N Takahashi; C L Arocha-Piñango; S Rodriguez; H Nagy; J L Perez-Requejo
Journal:  J Clin Invest       Date:  1992-07       Impact factor: 14.808

4.  Molecular basis of fibrinogen Naples associated with defective thrombin binding and thrombophilia. Homozygous substitution of B beta 68 Ala----Thr.

Authors:  J Koopman; F Haverkate; S T Lord; J Grimbergen; P M Mannucci
Journal:  J Clin Invest       Date:  1992-07       Impact factor: 14.808

5.  Abnormal fibrinogens IJmuiden (B beta Arg14----Cys) and Nijmegen (B beta Arg44----Cys) form disulfide-linked fibrinogen-albumin complexes.

Authors:  J Koopman; F Haverkate; J Grimbergen; L Engesser; I Nováková; A F Kerst; S T Lord
Journal:  Proc Natl Acad Sci U S A       Date:  1992-04-15       Impact factor: 11.205

6.  A novel mutation in exon 2 of FGB caused by c.221G>T † substitution, predicting the replacement of the native Arginine at position 74 with a Leucine (p.Arg74Leu † ) in a proband from a Kurdish family with dysfibrinogenaemia and familial venous and arterial thrombosis.

Authors:  Abdul A Shlebak; Alexia D Katsarou; George Adams; Fiona Fernando
Journal:  J Thromb Thrombolysis       Date:  2017-02       Impact factor: 2.300
  6 in total

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