Dysfunctional endogenous FIX impairs prophylaxis in a mouse hemophilia B model

Author:

Cooley Brian1,Broze George J.2,Mann David M.3,Lin Feng-Chang4,Pedersen Lee G.5,Stafford Darrel W.6

Affiliation:

1. Department of Pathology and Laboratory Medicine, University of North Carolina, Chapel Hill, NC;

2. Department of Medicine, Division of Medicine, Washington University in St. Louis, St. Louis, MO;

3. Mann BioConsulting, Gaithersburg, MD;

4. Department of Biostatistics,

5. Department of Chemistry, and

6. Department of Biology and Pathology, University of North Carolina at Chapel Hill, Chapel Hill, NC

Abstract

Key Points An endogenous, dysfunctional (CRM+) FIX molecule affects prophylactic FIX efficacy. Recovery studies indicate the amount of extravascular, Col4-bound FIX is several fold greater than the FIX in plasma.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Reference38 articles.

1. The binding of factor IXa to cultured bovine aortic endothelial cells. Induction of a specific site in the presence of factors VIII and X;Stern;J Biol Chem,1985

2. Binding of factors IX and IXa to cultured vascular endothelial cells;Stern;Proc Natl Acad Sci USA,1983

3. Identification of the endothelial cell binding site for factor IX;Cheung;Proc Natl Acad Sci USA,1996

4. Mammalian collagen IV;Khoshnoodi;Microsc Res Tech,2008

5. In vivo evidence of intravascular binding sites for coagulation factor IX;Stern;Br J Haematol,1987

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