Targeting anticoagulant protein S to improve hemostasis in hemophilia

Author:

Prince Raja12,Bologna Luca12,Manetti Mirko3ORCID,Melchiorre Daniela4,Rosa Irene3,Dewarrat Natacha12,Suardi Silvia5,Amini Poorya6,Fernández José A.7,Burnier Laurent7ORCID,Quarroz Claudia12,Reina Caro Maria Desiré12,Matsumura Yasuhiro8,Kremer Hovinga Johanna A.12ORCID,Griffin John H.7,Simon Hans-Uwe6ORCID,Ibba-Manneschi Lidia3,Saller François9ORCID,Calzavarini Sara12,Angelillo-Scherrer Anne12ORCID

Affiliation:

1. Department of Hematology and Central Hematology Laboratory, Inselspital, Bern University Hospital, and

2. Department of Clinical Research, University of Bern, Bern, Switzerland;

3. Department of Experimental and Clinical Medicine, Section of Anatomy and Histology, and

4. Department of Experimental and Clinical Medicine, Section of Internal Medicine, Rheumatology Unit, Careggi University Hospital, University of Florence, Florence, Italy;

5. Vetsuisse, Institute of Animal Pathology, Comparative Pathology Platform, and

6. Institute of Pharmacology, University of Bern, Bern, Switzerland;

7. Department of Molecular and Experimental Medicine, Scripps Research Institute, La Jolla, CA;

8. Division of Developmental Therapeutics, Research Centre for Innovative Oncology, National Cancer Centre Hospital East, Chiba, Japan; and

9. INSERM UMR-S 1176, Université Paris-Sud, Université Paris-Saclay, Le Kremlin-Bicêtre, France

Abstract

Key Points Targeting anticoagulant protein S rebalances coagulation in hemophilia. Protein S in joints is a novel pathophysiological contributor to hemarthrosis and a potential therapeutic target in hemophilia.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Reference47 articles.

1. Haemophilias A and B;Bolton-Maggs;Lancet,2003

2. Hemophilia A;Hoyer;N Engl J Med,1994

3. The hemophilias—from royal genes to gene therapy;Mannucci;N Engl J Med,2001

4. Modern haemophilia care;Berntorp;Lancet,2012

5. 2017 clinical trials update: innovations in hemophilia therapy;Hartmann;Am J Hematol,2016

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