Insertion Loop 256−268 in Coagulation Factor IX Restricts Enzymatic Activity in the Absence but Not in the Presence of Factor VIII†

Biochemistry ◽  
2000 ◽  
Vol 39 (25) ◽  
pp. 7398-7405 ◽  
Author(s):  
Joost A. Kolkman ◽  
Koen Mertens
Keyword(s):  
Enzymatic Activity ◽  
Factor Viii ◽  
Coagulation Factor ◽  
Factor Ix ◽  
Coagulation Factor Ix

The Interface between the EGF2 Domain and the Protease Domain in Blood Coagulation Factor IX Contributes to Factor VIII Binding and Factor X Activation

Biochemistry ◽  
2006 ◽  
Vol 45 (35) ◽  
pp. 10777-10785 ◽  
Author(s):  
Caroline Fribourg ◽  
Alexander B. Meijer ◽  
Koen Mertens
Keyword(s):  
Factor Viii ◽  
Blood Coagulation ◽  
Coagulation Factor ◽  
Factor Ix ◽  
Factor X ◽  
Protease Domain ◽  
Coagulation Factor Ix ◽  
Factor X Activation

scholarly journals Enhancement of the enzymatic activity of activated coagulation factor IX by anti-factor IX antibodies

2008 ◽  
Vol 6 (2) ◽  
pp. 315-322 ◽  
Author(s):  
F. SCHEIFLINGER ◽  
M. DOCKAL ◽  
J. ROSING ◽  
R. J. KERSCHBAUMER
Keyword(s):  
Enzymatic Activity ◽  
Coagulation Factor ◽  
Factor Ix ◽  
Coagulation Factor Ix

scholarly journals Blood Coagulation Factor IX Residues Glu78and Arg94Provide a Link between Both Epidermal Growth Factor-like Domains That Is Crucial in the Interaction with Factor VIII Light Chain

1998 ◽  
Vol 273 (1) ◽  
pp. 222-227 ◽  
Author(s):  
Olivier D. Christophe ◽  
Peter J. Lenting ◽  
Joost A. Kolkman ◽  
George G. Brownlee ◽  
Koen Mertens
Keyword(s):  
Growth Factor ◽  
Epidermal Growth Factor ◽  
Factor Viii ◽  
Blood Coagulation ◽  
Light Chain ◽  
Coagulation Factor ◽  
Factor Ix ◽  
Coagulation Factor Ix ◽  
Epidermal Growth

scholarly journals Cleavage at Arginine 145 in Human Blood Coagulation Factor IX Converts the Zymogen into a Factor VIII Binding Enzyme

1995 ◽  
Vol 270 (25) ◽  
pp. 14884-14890 ◽  
Author(s):  
Peter J. Lenting ◽  
Hans ter Maat ◽  
Patrick P. F. M. Clijsters ◽  
Marie-José S. H. Donath ◽  
Jan A. van Mourik ◽  
...  
Keyword(s):  
Factor Viii ◽  
Blood Coagulation ◽  
Human Blood ◽  
Coagulation Factor ◽  
Factor Ix ◽  
Coagulation Factor Ix ◽  
Human Blood Coagulation

scholarly journals Gene Therapy in Hemophilia: Recent Advances

2021 ◽  
Vol 22 (14) ◽  
pp. 7647
Author(s):  
E. Carlos Rodríguez-Merchán ◽  
Juan Andres De Pablo-Moreno ◽  
Antonio Liras
Keyword(s):  
Gene Therapy ◽  
Adverse Events ◽  
Factor Viii ◽  
Coagulation Factor ◽  
Cost Savings ◽  
Factor Ix ◽  
Clotting Factors ◽  
Advanced Therapies ◽  
Potential Benefits

Hemophilia is a monogenic mutational disease affecting coagulation factor VIII or factor IX genes. The palliative treatment of choice is based on the use of safe and effective recombinant clotting factors. Advanced therapies will be curative, ensuring stable and durable concentrations of the defective circulating factor. Results have so far been encouraging in terms of levels and times of expression using mainly adeno-associated vectors. However, these therapies are associated with immunogenicity and hepatotoxicity. Optimizing the vector serotypes and the transgene (variants) will boost clotting efficacy, thus increasing the viability of these protocols. It is essential that both physicians and patients be informed about the potential benefits and risks of the new therapies, and a register of gene therapy patients be kept with information of the efficacy and long-term adverse events associated with the treatments administered. In the context of hemophilia, gene therapy may result in (particularly indirect) cost savings and in a more equitable allocation of treatments. In the case of hemophilia A, further research is needed into how to effectively package the large factor VIII gene into the vector; and in the case of hemophilia B, the priority should be to optimize both the vector serotype, reducing its immunogenicity and hepatotoxicity, and the transgene, boosting its clotting efficacy so as to minimize the amount of vector administered and decrease the incidence of adverse events without compromising the efficacy of the protein expressed.

scholarly journals Human blood coagulation factor IX. Purification, properties, and mechanism of activation by activated factor XI.

1978 ◽  
Vol 253 (17) ◽  
pp. 5946-5951 ◽  
Author(s):  
B. Osterud ◽  
B.N. Bouma ◽  
J.H. Griffin
Keyword(s):  
Blood Coagulation ◽  
Human Blood ◽  
Coagulation Factor ◽  
Factor Ix ◽  
Factor Xi ◽  
Coagulation Factor Ix ◽  
Human Blood Coagulation
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