Abstract |
Hemophilia is a severe bleeding disorder treated by infusion of the missing blood coagulation protein, factor VIII or factor IX. The discovery and characterization of the anticoagulant protein tissue factor pathway inhibitor ( TFPI) led to the realization that inhibition of TFPI activity could restore functional hemostasis through the extrinsic blood coagulation pathway in a manner that does not require the activity of factors VIII or IX. There are currently several therapeutic agents that inhibit TFPI in development for treatment of hemophilia. A comprehensive understanding of TFPI structure, biochemistry, and cellular expression is necessary to understand how it modulates bleeding in hemophilia and the physiological impact of therapeutic agents targeting TFPI.
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Authors | Julie A Peterson, Susan A Maroney, Alan E Mast |
Journal | Thrombosis research
(Thromb Res)
Vol. 141 Suppl 2
Pg. S28-30
(May 2016)
ISSN: 1879-2472 [Electronic] United States |
PMID | 27207418
(Publication Type: Journal Article, Review)
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Copyright | Copyright © 2016 Elsevier Ltd. All rights reserved. |
Chemical References |
- Lipoproteins
- lipoprotein-associated coagulation inhibitor
- Factor VIII
- Factor IX
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Topics |
- Animals
- Blood Coagulation
(drug effects)
- Drug Discovery
(methods)
- Factor IX
(metabolism)
- Factor VIII
(metabolism)
- Hemophilia A
(blood, drug therapy, metabolism)
- Hemophilia B
(blood, drug therapy, metabolism)
- Hemorrhage
(blood, drug therapy, metabolism)
- Humans
- Lipoproteins
(analysis, antagonists & inhibitors, metabolism)
- Molecular Targeted Therapy
(methods)
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