Article
Mutations Accounting for Congenital Fibrinogen Disorders: An Update.
Seminars in thrombosis and hemostasis - 1 Nov 2022
Richard Maxime, Celeny David, Neerman-Arbez Marguerite
Abstract excerpt
Fibrinogen is a complex protein that plays a key role in the blood clotting process. It is a hexamer composed of two copies of three distinct chains: Aα, Bβ, and γ encoded by three genes, FGA, FGB, and FGG, clustered on the long arm of chromosome 4. Congenital fibrinogen disorders (CFDs) are divided into qualitative deficiencies (dysfibrinogenemia, hypodysfibrinogenemia) in which the mutant fibrinogen molecule is...
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