Article
Hereditary Hypofibrinogenemia with Hepatic Storage.
International journal of molecular sciences - 22 Oct 2020
Asselta Rosanna, Paraboschi Elvezia Maria, Duga Stefano
Abstract excerpt
Fibrinogen is a 340-kDa plasma glycoprotein constituted by two sets of symmetrical trimers, each formed by the Aα, Bβ, and γ chains (respectively coded by the FGA, FGB, and FGG genes). Quantitative fibrinogen deficiencies (hypofibrinogenemia, afibrinogenemia) are rare congenital disorders characterized by low or unmeasurable plasma fibrinogen antigen levels. Their genetic basis is represented by mutations within...
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