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Blood Coagulation and Fibrinolytic Factors: Poster II

Program: Oral and Poster Abstracts
Type: Poster
Sunday, December 5, 2010: 6:00 PM-8:00 PM
Hall A3/A4 (Orange County Convention Center)
Protein S Regulates Factor IXa/VIIIa Activity Independent of Activated Protein C

Rinku Majumder, PhD

Biochemistry & Biophysics, University of North Carolina at Chapel Hill, Chapel Hill, NC

Poster Board Number: II-76
The Synonymous V107V Mutation In Factor IX Is Not So Silent and May Cause Hemophilia B In Patients

Chava Kimchi-Sarfaty, Ph.D.1, Vijaya L. Simhadri, Ph.D.2*, David Kopelman3*, Adam Friedman4*, Nathan C. Edwards3*, Atiq Javaid4*, Chinyere Okunji4*, Anton A. Komar, Ph.D.5*, Zuben E. Sauna, Ph.D.3* and Nobuko H. Katagiri, Ph.D.3*

1Center for Biologics Evaluation and Research, Division of Hematology, Food and Drug Administration , Bethesda, MD
2Center for Biologics Evaluation and Reearch, Division of Hematology, Food and Drug Administration, Bethesda, MD
3Center for Biologics Evaluation and Research, Division of Hematology, Food and Drug Administration, Bethesda, MD
4Division of Hematology, CBER, Food and Drug Administration (FDA), Bethesda, MD
5Cleveland State University, Cleveland, OH

Poster Board Number: II-77
De Novo Synthesis & Storage of Human Factor VIII In Platelets Reduces Bleeding In Canine Hemophilia A

Lily M. Du, MD1*, Helen W.G. Franck, BS2*, Elizabeth P. Merricks, PhD2*, Paquita Nurden, MD3*, Eric S. Jensen, DVM4*, Sandra L. Haberichter, PhD1, Troy B. Hawkins, PhD5*, Paula M. Jacobi, BS1*, Juan Fang, MD1*, Timothy C. Nichols, MD6, Sevasti B. Koukouritaki, PhD1*, Qizhen Shi, MD, PhD1, Robert R. Montgomery, MD1, Kenneth Cornetta, MD5, Alan T. Nurden, PhD7* and David A. Wilcox, PhD1

1Dept of Pediatrics, Medical College of Wisconsin, Children's Research Institute, MACC Fund Research Center, Blood Research Institute, Milwaukee, WI
2Francis Owen Blood Research Laboratory, Dept of Pathology and Laboratory Medicine, University of North Carolina, Chapel Hill, NC
3C R P P / P T I B, Hôpital Xavier Arnozan, Pessac, France
4Biomedical Resource Center, Medical College of Wisconsin
5Dept of Medical and Molecular Genetics, Indiana University School of Medicine, Indianapolis, IN
6Dept of Pathology and Laboratory Medicine, University of North Carolina, Chapel Hill, NC
7PTIB, Centre de référence des Pathologies Plaquettaires, Pessac, France

Poster Board Number: II-78
Critical Role of Immobilized Factor VIII In Solid-Phase Blood Coagulation During Mural Thrombogenesis Under Whole Blood Flow Conditions

Masaaki Doi, MD1*, Mitsuhiko Sugimoto, MD, PhD1*, Hideto Matsui, MD, PhD1, Tomoko Matsumoto2* and Midori Shima, MD, PhD3

1Regulatory Medicine for Thrombosis, Nara Medical University, Kashihara, Nara, Japan
2Pediatrics, Nara Medical University, Kashihara, Nara, Japan
3Department of Pediatrics, Nara Medical University, Kashihara, Nara, Japan

Poster Board Number: II-79
Functional Analysis of a Peptide Sequence (Thr323 - Cys337) In ADAMTS13 Disintegrin-Like Domain Revealed Two Discontinuous Sequences Involved In the Interaction with VWF

Atsuko Igari1*, Takanori Moriki2*, Terumichi Nakagawa1*, Yusuke Yamaguchi1* and Mitsuru Murata1

1Laboratory Medicine, Keio University School of Medicine, Tokyo, Japan
2Health Center, Keio University, Tokyo, Japan

Poster Board Number: II-80
The Important Role of Von Willebrand Factor In Platelet-Derived FVIII Gene Therapy of Murine Hemophilia A In the Presence of Inhibitors

Qizhen Shi, MD, PhD, Erin L. Kuether*, Jocelyn A. Schroeder*, Scot A. Fahs*, David A. Wilcox, PhD and Robert R. Montgomery, MD

Medical College of Wisconsin, Blood Research Institute, Children's Research Institute, Milwaukee, WI

Poster Board Number: II-81
Humoral and Anaphylactic Responses to Factor IX In Murine Hemophilia B Are Genotype Dependent and Can Be Reversed by Hepatic Gene Transfer

David Markusic, PhD1*, Sushrusha Nayak, PhD2* and Roland W Herzog, PhD1

1Pediatrics/CMT, University of Florida, Gainesville, FL
2Pediatrics, University of Florida, Gainesville, FL

Poster Board Number: II-82
The Role of Amino Acids 700-701 of the Factor Va Heavy Chain During Prothrombin Activation by Factor Xa

Jamila Hirbawi, PhD1*, Paul Y Kim, PhD2*, Michael E. Nesheim, PhD3 and Michael Kalafatis, PhD4

1Molecular Cardiology, Cleveland Clinic, Cleveland, OH
2Biochemistry, Queen's University, Kingston, ON, Canada
3Dept. of Biochemistry, Queen's University, Kingston, ON, Canada
4Dept. of Chemistry, Cleveland State University, Cleveland, OH

Poster Board Number: II-83
Ideal Amphipathic Peptdes Coupled to Nanofibrous Micropheres Reduce Hemorrhage In Vivo

Catherine Lemarie, Dr.1*, Jianqiu Wu, Dr2*, Jake Barralet, Dr.3* and Mark Blostein, Dr.4

1Medicine, Jewish General Hospital, McGill University, Monreal, QC, Canada
2Medicine, Lady Davis Institute for Medical Research, McGill University, Montreal, QC, Canada
3Dentistry, McGill University, Montreal, QC
4Jewish General Hospital, Montreal, QC, Canada

Poster Board Number: II-84
Understanding Ectopically Expressed Factor VIII (F8) In Megakaryocytes: Implications for Optimum Platelet-Delivered F8 Activity for Gene Therapy

Teshell K Greene, B.S1*, Denise E. Sabatino, Ph.D.2, Nicholas P Iacobelli, B.S1*, Li Zhai, MD3*, Steven W. Pipe, MD4, M. Anna Kowalska, PhD3, Rodney M. Camire, PhD5 and Valder Arruda, MD, PhD6

1Hematology, Children's Hospital of Philadelphia, Philadelphia, PA
2Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA
3Division of Hematology, The Children's Hospital of Philadelphia, Philadelphia, PA
4Pediatrics, University of Michigan, Ann Arbor, MI
5Hematology, The Children's Hospital of Philadelphia, Philadelphia, PA
6Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA

Poster Board Number: II-85
B-Cell Depletion Using IgG1 Anti-CD20 Spares MZ B Cells and Promotes Tolerance to Human FVIII In a Murine Model of Hemophilia A

Ai-Hong Zhang, Ph.D.1*, Jonathan Skupsky, PhD1* and David W. Scott, PhD2

1Center for Vascular and Inflammatory Diseases, University of Maryland School of Medicine, Baltimore, MD
2Depts. of Surgery & Microbiology-Immunology, University of Maryland School of Medicine, Baltimore, MD

Poster Board Number: II-86
De-Immunization of Immunodominant HLA-DR Epitopes In the C2 Domain of Factor VIII 

Chang Song, PhD1*, Leonard Moise, Ph.D.2*, Ryan Tassone3*, Elizabeth McClaine3*, William D. Martin3*, Annie S. DeGroot, M.D.2* and David W. Scott, PhD4

1Center for Vascular and Inflammatory Diseases, University of Maryland School of Medicine, Baltimore, MD
2EpiVax, Inc. and Institute for Immunology and Informatics, University of Rhode Island, Providence, RI
3EpiVax, Inc., Providence, RI
4Depts. of Surgery & Microbiology-Immunology, University of Maryland School of Medicine, Baltimore, MD

Poster Board Number: II-87
Low VWF:RCo In Subjects with VWF Polymorphisms D1472H and P1467S Due to Decreased Binding of Ristocetin to the VWF A1 DomainClinically Relevant Abstract

Veronica H Flood, MD1, Jeffrey S Wren2*, Sandra L Haberichter, PhD1, Kenneth D Friedman, MD3, Daniel B. Bellissimo, PhD3, Joan Cox Gill, MD1, Raymond G Hoffmann, PhD1*, J. Paul Scott, MD1* and Robert R Montgomery, MD4

1Pediatrics, Medical College of Wisconsin, Milwaukee, WI
2Blood Center of SE Wisconsin, Milwaukee, WI
3BloodCenter of Wisconsin, Milwaukee, WI
4TS Zimmerman Program for the Molecular and Clinical Biology of VWD, Blood Research Institute, Medical College of Wisconsin, Milwaukee, WI

Poster Board Number: II-88
N1922S Mutation In the Factor VIII A3 Domain Produces a Rate-Limiting, Domain-Specific Folding Defect Leading to Hyposecretion of a Functional Protein

Ryan J. Summers, B.S.1*, Shannon L. Meeks, MD2, John F. Healey, B.S.2*, Harrison C. Brown, BBA2*, Ernest T Parker, BA2*, Christopher Doering, PhD2, Christine L. Kempton, MD, MSc3* and John (Pete) S. Lollar III, MD2

1Emory University School of Medicine, Atlanta, GA
2Aflac Cancer Center and Blood Disorders Service, Children's Healthcare of Atlanta/Emory University, Atlanta, GA
3Aflac Cancer Center and Blood Disorders Service and Department of Hematology/Medical Oncology, Emory University, Atlanta, GA

Poster Board Number: II-89
Identification and Cloning of Novel Mutations In a Compound Heterozygous Factor V Deficient PatientClinically Relevant Abstract

Kimberley Talbot, MPhil1*, Jina Song, PhD1*, Lily Eghdami2*, Jessica Tamura-Wells3*, Jeff Hewitt, PhD4*, Linda M Vickars, MD, FRCP(C)5*, Cedric John Carter, MD2, Ross T.A. MacGillivray, PhD4* and Ed L.G. Pryzdial6

1Research and Development, Canadian Blood Services, Centre for Blood Research/Pathology and Laboratory Medicine, University of British Columbia, Vancouver, BC, Canada
2Pathology and Laboratory Medicine/Centre for Blood Research, University of British Columbia, Vancouver, BC, Canada
3Research and Development, Canadian Blood Services, Centre for Blood Research, University of British Columbia, Vancouver, BC, Canada
4Biochemistry and Molecular Biology/Centre for Blood Research, University of British Columbia, Vancouver, BC, Canada
5Hematology, St. Paul's Hospital and the University of British Columbia, Vancouver, BC, Canada
6Canadian Blood Services, Research and Development Department, and University of British Columbia, Centre for Blood Research, Department of Pathology and Laboratory Medicine, Vancouver, BC, Canada

Poster Board Number: II-90
Non-Catalytic Domains of ADAMTS13 Can Redirect the Substrate Specificity of ADAMTS5

Weiqiang Gao, MD, PhD1*, Elodee Tuley, BSc1* and J. Evan Sadler, MD, PhD2

1Department of Medicine, Washington Univ. School of Medicine, Saint Louis, MO
2Department of Medicine, Washington University Medical School, Saint Louis, MO

Poster Board Number: II-91
Endoplasmic Reticulum to Mitochondria Ca2+ Signaling Inhibits Factor VIII Secretion and Mediates Oxidative Stress and Apoptosis Upon Factor VIII Expression 

Jyoti Malhotra1*, Mon Xiong1*, Hongzhi Miao, MS2*, Steven Pipe, MD2 and Randal J. Kaufman, PhD3

1Biological Chemistry, University of Michigan, Ann Arbor, MI
2Pediatrics & Communicable Diseases, University of Michigan, Ann Arbor, MI
3Univ. of Michigan Medical Ctr., Howard Hughes Medical Inst., Ann Arbor, MI

Poster Board Number: II-92
Reconstitution of Recombinant Factor VIII In fVIII-/- mice Restores Von Willebrand Factor Homeostasis

Wenjing Cao, M.D., Ph.D.1*, Denise E Sabatino, Ph.D.2, Ekaterina Altynova, B.S.2* and X. Long Zheng, M.D., Ph.D.1*

1Department of Pathology and Laboratory Medicine, The Children's Hospital of Philadelphia, Philadelphia, PA
2Department of Genetics, The University of Pennsylvania, Philadelphia, PA

Poster Board Number: II-93
BAY 94-9027, a PEGylated Recombinant Human FVIII, Shows Less Immunogenicity Compared to Un-PEGylated Recombinant FVIII

Inge A Ivens, PhD., D.A.B.T.1*, Ruprecht Zierz, PhD2*, Jesper Haaning, PhD1* and Thomas McDonald, MPH, PhD1*

1Bayer HealthCare Pharmaceuticals, Richmond, CA
2Bayer Schering Pharma, Berlin, Germany

Poster Board Number: II-94
Meizothrombin Is Unexpectedly Zymogen-Like: Its Slow Conversion to Proteinase Dominates Thrombin Production by Prothrombinase

Harlan Bradford, BA* and Sriram Krishnaswamy

Hematology, The Children's Hospital of Philadelphia, Philadelphia, PA

Poster Board Number: II-95
Endogenous Expression of Functional Factor VIII by Human Mesenchymal Stem Cells In Culture

Chad Sanada*, Evan J Colletti*, Melisa Soland*, Chung-Jung Kuo*, Christopher D Porada, PhD and Graca Almeida-Porada, MD, PhD

Animal Biotechnology, University of Nevada, Reno, Reno, NV

Poster Board Number: II-96
Association of Quantitative and Qualitative Abnormalities of Von Willebrand Factor and Risk of Death In Hemodialysis PatientsClinically Relevant Abstract

Rachel Holden, MD1*, Angie Tuttle1*, Francis MacLeod1*, Toni Burbidge1*, Carol Hegadorn2* and Paula D. James, MD3

1Queen's University, Kingston, ON, Canada
2Pathology and Molecular Medicine, Queen's University, Kingston, ON, Canada
3Medicine, Queen's University, Kingston, ON, Canada

Poster Board Number: II-97
Investigation of the Role of Copy Number Variation In the Pathogenesis of Type 1 Von Willebrand Disease

Daniel J. Hampshire, PhD1*, Lisa D. Bloomer, MSc1*, Ahlam M. Al-Buhairan, PhD1*, Rachael E. Coyle1*, Raymon N. C. P. Vijzelaar, MSc2*, David Lillicrap, MD3*, Paula D. James, MD4, Jeroen C. J. Eikenboom, MD, PhD5, Reinhard Schneppenheim, MD, PhD6, Francesco Rodeghiero, MD7*, Robert R. Montgomery, MD8, Ian R. Peake, PhD1 and Anne C. Goodeve, PhD1*

1Haemostasis Research Group, University of Sheffield, Sheffield, United Kingdom
2MRC-Holland b.v., Amsterdam, Netherlands
3Department of Pathology and Molecular Medicine, Queen's University, Kingston, ON, Canada
4Department of Medicine, Queen's University, Kingston, ON, Canada
5Department of Thrombosis and Haemostasis, Leiden University Medical Center, Leiden, Netherlands
6Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
7Department of Hematology, San Bortolo Hospital, Vicenza, Italy
8TS Zimmerman Program for the Molecular and Clinical Biology of VWD, Blood Research Institute, Medical College of Wisconsin, Milwaukee, WI

Poster Board Number: II-98
Molecular Mechanism Underlying the Need for Three Cleavage Sites within the B-Domain to Activate Factor V by Thrombin

Roisin Walshe, BS1*, Mettine H.A. Bos, PhD2* and Rodney M. Camire, PhD1

1Hematology, Children's Hospital of Philadelphia, Philadelphia, PA
2Hematology, The Children's Hospital of Philadelphia, Philadelphia, PA

Poster Board Number: II-99
Asparagine-Linked Glycosylation at the NH2-Terminus Does Not Influence Secretion or Function of Coagulation Factors V and VIII

Sundar Rajan Selvaraj, PhD1 and Steven W Pipe, MD2

1Pediatrics & Communicable Diseases, University of Michigan, Ann Arbor, MI
2Pediatrics and Communicable Diseases, Mott Children's Hospital, Ann Arbor, MI

Poster Board Number: II-100
Amino Acid Region 1000-1008 of Coagulation Factor V Is a Dynamic Regulator for the Emergence of Procoagulant Activity

Joesph R Wiencek, B.A.1*, Jamila Hirbawi, PhD2*, Mahesheema Na, MSc1* and Michael Kalafatis, PhD1

1Chemistry, Cleveland State University, Cleveland, OH
2Molecular Cardiology, Cleveland Clinic, Cleveland, OH

Poster Board Number: II-101
Discovery and Characterization of An Anti-APC Aptamer for Use In Hemophilia

Patricia G.M. Wagner1*, Michael C. Schwartz1*, Kathleen E. McGinness1*, Ryan M. Genga1*, Jeffrey C. Kurz, PhD1, Emily K. Waters, PhD1 and Robert Schaub, PhD2

1Archemix Corporation, Cambridge, MA
2Preclinical Discovery, Archemix Corporation, Cambridge, MA

Poster Board Number: II-102
The Diversity of the Immune Response to the A2 Domain of Human Factor VIII In a Murine Hemophilia A Model

John F. Healey, B.S.*, John (Pete) S. Lollar III, M.D., Ernest T Parker, B.A.* and Shannon Meeks, M.D.

Aflac Cancer Center and Blood Disorders Service, Children's Healthcare of Atlanta/Emory University, Atlanta, GA

Poster Board Number: II-103
Differential Engagement of the Unfolded Protein Response by Human and Porcine Factor VIII

Harrison C. Brown, BBA1*, Bagirath Gangadharan, MS1* and Christopher B Doering, PhD2

1Emory University, Atlanta, GA
2Emory Childrens Center Pediatrics, Emory University, Atlanta, GA

Poster Board Number: II-104

*signifies non-member of ASH