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321.O3.8 Coagulation Cofactors and Von Willebrand Factor

Monday, December 8, 2008: 3:30 PM-5:30 PM
3014-3016-3018 - West (Moscone Center)
Moderators:
Judith Lin, MD and Reinhard Schneppenheim, MD
3:30 PM
Platelet-Derived Factor Va Expressed on the Surface of the Activated Platelet Is GPI-Anchored

Jeremy P Wood1*, Ammon M. Fager1*, Jay R. Silveira1* and Paula B. Tracy, PhD2

1Biochemistry, University of Vermont, Burlington, VT
2University of Vermont, Burlington, VT

3:45 PM
Evolutionary Adaptation of Factor V from the Venom of the Common Brown Snake into a Potent Procoagulant

Mettine H.A. Bos, Ph.D.1, Michael Boltz1*, Liam St. Pierre, Ph.D.2*, John de Jersey, Ph.D.3*, Paul P Masci, Ph.D.3*, Martin L Lavin, Ph.D.4* and Rodney M. Camire, Ph.D.1

1Hematology, Children's Hospital of Philadelphia, Philadelphia, PA
2University of Queensland Centre for Clinical Research, Brisbane, Australia
3Faculty of Health Sciences, University of Queensland, Brisbane, Australia
4University of Queensland Centre for Clinical Research/QRxPharma Ltd, Brisbane/Sydney, Australia

4:00 PM
Factor VIII C1 Domain Residues 2092-2093 Participate in Membrane Binding

Henriet Meems1*, Alexander B Meijer1*, Dave Cullinan2*, Koen Mertens, PhD1 and Gary E. Gilbert2*

1Dept Plasma Proteins, Sanquin Blood Supply Foundation, Amsterdam, Netherlands
2VA Boston Healthcare System, Harvard Medical School, Boston, MA

4:15 PM
Intersection of Mechanisms of Type 2A Von Willebrand Disease through Defects in Von Willebrand Factor Multimerization, Secretion, ADAMTS-13 Susceptibility, and Regulated Storage

Paula M. Jacobi1*, Joan Cox Gill, MD2, Kenneth D. Friedman, MD3, Robert R. Montgomery, MD4 and Sandra L. Haberichter, PhD5

1Pediatrics, Medical College of Wisconsin, Milwaukee, WI
2Bleeding Disorders, Comprehensive Center for, Milwaukee, WI
3Blood Research Institute, Medical College of Wisconsin, TS Zimmerman Program for the Molecular and Clinical Biology of VWD, Milwaukee, WI
4TS Zimmerman Program for the Molecular and Clinical Biology of VWD, Blood Research Institute, Medical College of Wisconsin, Milwaukee, WI
5Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI

4:30 PM
Force Dependent Structural Changes of the Von Willebrand Factor A2 Domain Are Required for VWF Cleavage by ADAMTS13

Reinhard Schneppenheim, MD, PhD1, Carsten Baldauf, PhD2*, Tobias Obser1*, Ulrich Budde, MD3, Antje Pieconka3*, Sonja Schneppenheim, MD3* and Frauke Graeter, PhD2*

1Pediatric Hem. & Onc., University Med. Ctr., Hamburg-Eppendorf, Hamburg, Germany
2CAS-MPG Partner Institute for Computational Biology, Shanghai Institutes for Biological Sciences, Shanghai, China
3Coagulation Laboratory, Aesculabor Hamburg, Hamburg, Germany

4:45 PM
Factor VIII and Platelets Cooperatively Accelerate Proteolytic Cleavage of Von Willebrand Factor by ADAMTS13 Under Shear Stress

Christopher G. Skipwith*, Wenjing Cao* and X. Long Zheng

Pathology and Laboratory Medicine, Children's Hospital of Philadelphia and University of Pennsylvania, Philadelphia, PA

5:00 PM
Suppression of the Immune Response to Human FVIII with FVIII Transgene Modified Tolerogenic Dendritic Cells in Hemophilic Mice

Rui-Jun Su, Ph.D*, Angela Epp*, Xiaoping Wu, Ph.D* and Neil Josephson, MD

Research, Puget Sound Blood Center, Seattle, WA

5:15 PM
A Novel Role for Factor VIII and Other Coagulation Factors in Organ Size Control

Anna Aronovich1*, Dalit Tchorsh1*, Elias Shezen1*, Chava Rosen1*, Yael Klionsky1*, Sivan Cohen1*, Orna Tal1*, Uri Martinovich2*, Helena Katchman1*, Smadar Eventov-Friedman1*, Ninette Amariglio, PhD3, Jasmine Jacob-Hirsch4*, Gideon Rechavi, MD, PhD3* and Yair Reisner1

1Immunology, Weizmann Institute of Science, Rehovot, Israel
2Israel National Hemophilia Center, Sheba Medical Center, Tel Hashomer, Israel
3Cancer Research Center, Sheba M.C., Tel-Hashomer, Israel
4Cancer Research Center, Sheba M.C., Tel-Hashomer, Israel, Ramat-Gan, Israel

*signifies non-member of ASH