Orphan designation: Recombinant human coagulation factor VIII Fc - von Willebrand factor - XTEN fusion protein, Treatment of haemophilia A

2019 ◽  
Author(s):  
Author(s):  
И.В. Куртов ◽  
Е.С. Фатенкова ◽  
Н.А. Юдина ◽  
А.М. Осадчук ◽  
И.Л. Давыдкин

Болезнь Виллебранда (БВ) может представлять определенные трудности у рожениц с данной патологией. Приведены 2 клинических примера использования у женщин с БВ фактора VIII свертывания крови с фактором Виллебранда, показана эффективность и безопасность их применения. У одной пациентки было также показано использование фактора свертывания крови VIII с фактором Виллебранда во время экстракорпорального оплодотворения. Von Willebrand disease presents a certain hemostatic problem among parturients. This article shows the effectiveness and safety of using coagulation factor VIII with von Willebrand factor for the prevention of bleeding in childbirth in 2 patients with type 3 von Willebrand disease. In one patient, the use of coagulation factor VIII with von Willebrand factor during in vitro fertilization was also shown.


2021 ◽  
Vol 5 (3) ◽  
pp. 812-822
Author(s):  
Philip M. Zakas ◽  
Christopher W. Coyle ◽  
Anja Brehm ◽  
Marion Bayer ◽  
Barbara Solecka-Witulska ◽  
...  

Abstract Ancestral sequence reconstruction provides a unique platform for investigating the molecular evolution of single gene products and recently has shown success in engineering advanced biological therapeutics. To date, the coevolution of proteins within complexes and protein–protein interactions is mostly investigated in silico via proteomics and/or within single-celled systems. Herein, ancestral sequence reconstruction is used to investigate the molecular evolution of 2 proteins linked not only by stabilizing association in circulation but also by their independent roles within the primary and secondary hemostatic systems of mammals. Using sequence analysis and biochemical characterization of recombinant ancestral von Willebrand factor (VWF) and coagulation factor VIII (FVIII), we investigated the evolution of the essential macromolecular FVIII/VWF complex. Our data support the hypothesis that these coagulation proteins coevolved throughout mammalian diversification, maintaining strong binding affinities while modulating independent and distinct hemostatic activities in diverse lineages.


PLoS ONE ◽  
2017 ◽  
Vol 12 (8) ◽  
pp. e0183590 ◽  
Author(s):  
Klytaimnistra Kiouptsi ◽  
Alexandra Grill ◽  
Amrit Mann ◽  
Mareike Döhrmann ◽  
Maren Lillich ◽  
...  

1991 ◽  
Vol 180 (1) ◽  
pp. 231-237 ◽  
Author(s):  
Peter R. Ganz ◽  
Judith S. Atkins ◽  
Douglas S. Palmer ◽  
Anil K. Dudani ◽  
Sophia Hashemi ◽  
...  

2015 ◽  
Vol 103 (2) ◽  
pp. 189-195 ◽  
Author(s):  
Martin Mohren ◽  
Kathleen Jentsch-Ullrich ◽  
Michael Koenigsmann ◽  
Siegfried Kropf ◽  
Enrico Schalk ◽  
...  

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