Role of factor XIII in fibrin clot formation and effects of genetic polymorphisms

作者: Robert A. S. Ariëns , Thung-Shenq Lai , John W. Weisel , Charles S. Greenberg , Peter J. Grant

DOI: 10.1182/BLOOD.V100.3.743

关键词:

摘要: Factor XIII and fibrinogen are unusual among clotting factors in that neither is a serine protease. Fibrin the main protein constituent of blood clot, which stabilized by factor XIIIa through an amide or isopeptide bond ligates adjacent fibrin monomers. Many structural functional features fibrin(ogen) have been elucidated gene analysis, site-directed mutagenesis, x-ray crystallography. However, some molecular aspects involved complex processes insoluble formation vivo vitro remain unresolved. The findings relationship between fibrinogen, XIII, cardiovascular other thrombotic disorders focused much attention on these 2 proteins. Of particular interest associations common variations genes altered risk profiles for thrombosis. Although there debate regarding observations, implications our understanding clot therapeutic intervention may be major importance. In this review, we summarized recent structure function XIII. This followed review effects genetic polymorphisms structure/function their to disease.

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