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Publication : Synthesis of a mouse model of the dysfibrinogen Vlissingen/Frankfurt IV.

First Author  Hogan KA Year  2001
Journal  Ann N Y Acad Sci Volume  936
Pages  117-21 PubMed ID  11460467
Mgi Jnum  J:130183 Mgi Id  MGI:3771181
Doi  10.1111/j.1749-6632.2001.tb03498.x Citation  Hogan KA, et al. (2001) Synthesis of a mouse model of the dysfibrinogen Vlissingen/Frankfurt IV. Ann N Y Acad Sci 936:117-21
abstractText  The dysfibrinogen Vlissingen/Frankfurt IV is characterized as a deletion of Asn319 and Asp320 from the C-terminus of the gamma-chain of fibrinogen. This dysfibrinogen, which was identified in several family members that are all heterozygous for the in-frame 6-bp deletion, is associated with both venous and arterial thrombosis. Here, we describe the generation of a murine model of the V/F IV dysfibrinogen using gene targeting of mouse gamma-chain DNA. Preliminary analysis shows that the human and mouse variant fibrinogens are similar: analogous to the human V/F IV protein, the D1 fragment of the variant mouse fibrinogen is partially protected from digestion in the presence of calcium or Gly-Pro-Arg-Pro. These heterozygous mice provide the first opportunity to examine the association of thrombophilia and dysfibrinogenemia in a controlled genetic background.
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