Scientific article

Protein modelling to understand FGB mutations leading to congenital hypofibrinogenaemia

Published inHaemophilia, vol. 23, no. 4, p. 583-589
Publication date2017

Congenital hypofibrinogenaemia is a quantitative fibrinogen disorder characterized by proportionally decreased levels of functional and antigenic fibrinogen. Mutations accounting for quantitative fibrinogen disorders are relatively frequent in the conserved COOH-terminal globular domains of the γ and Bβ chains. The latter mutations are of particular interest since the Bβ-chain is considered the rate-limiting chain in the hepatic production of the fibrinogen hexamer.

Citation (ISO format)
CASINI, Alessandro et al. Protein modelling to understand FGB mutations leading to congenital hypofibrinogenaemia. In: Haemophilia, 2017, vol. 23, n° 4, p. 583–589. doi: 10.1111/hae.13190
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Article (Published version)
ISSN of the journal1351-8216

Technical informations

Creation09/11/2017 1:29:00 PM
First validation09/11/2017 1:29:00 PM
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