65th ASH Annual Meeting & Exposition: 321. Coagulation and Fibrinolysis: Basic and Translational: Poster II

Symposia: Coagulation and Fibrinolysis: Basic and Translational Oral and Poster Abstracts
Poster

Halls G-H (San Diego Convention Center)

Cryo-EM Structure of Coagulation Factor VIII Bound to a Patient-Derived Anti-A2 Domain Antibody Inhibitor

Kenneth C Childers, PhD¹^*, Jordan Vaughan, MS¹^*, John S. Lollar, MD², Christopher B Doering, PhD³, Carmen Helena Coxon, PhD⁴^* and P. Clint Spiegel Jr., PhD¹

¹Chemistry Department, Western Washington University, Bellingham, WA
²Emory University, Children's Center, Atlanta, GA
³Department of Pediatrics, Aflac Cancer and Blood Disorders Center, Emory University and Children's Healthcare of Atlanta, Atlanta, GA
⁴National Institute of Biological Standards and Control, Potters Bar, ENG, GBR

2599

Forms of Hereditary Angioedema That Are Independent of Kallikrein and Factor XIIa System

Maxim Litvak, MD, PhD¹^*, Stephen Kent Dickeson, PhD¹^*, Sunil Kumar, PhD¹^*, Dennis Phillips, PhD²^*, Ruby Law, PhD³^* and David Gailani, MD¹

¹Department of Pathology, Immunology and Microbiology, Vanderbilt University Medical Center, Nashville, TN
²Department of Chemistry, University of Georgia, Athens, GA
³Department of Biochemistry and Molecular Biology, Monash University, Calyton, AUS

2600

Analysis and Mechanism(s) of Hemophilia a Pain in Factor VIII Knockout Mice

Raghda T Fouda, MD/PhD¹^*, Donovan Alexander Argueta, PhD¹, Natalie Garcia¹^*, Yugal Goel, PhD¹^*, Graham J Velasco²^*, Kendall O'Daniel¹^*, Kristen Peterson¹^* and Kalpna Gupta, PhD^1,3

¹Hematology/Oncology Division, Department of Medicine, University of California, Irvine, CA
²Pathology Department, VA Long Beach Healthcare System, Long Beach, Long Beach, CA
³Department of Medicine, Division of Hematology, Oncology and Transplantation, University of Minnesota, Minneapolis, MN

2601

Liver Sinusoidal Endothelial Cells but Not Hepatocytes Express a Fluorescent Reporter Under the Endogenous F8 Promoter in a New Hemophilia a Mouse Model

Paige Patterson, DO, Radoslaw Kaczmarek, PhD^* and Roland W. Herzog, PhD

Indiana University School of Medicine, Indianapolis, IN

2602

Intravenous 3K3A-Activated Protein C Inhibits Murine Ocular Inflammation and Suppresses Ocular Choroidal Neovascularization

Tami Livnat^1,2^*, Yael Nisgav, Msc¹^*, José A Fernández, MD, PhD³^*, Ivan Budnik, MD, PhD⁴^*, Sarina Levy-Mendelovich, MD⁵, Dov Weinberger, MD¹^*, Gili Kenet, MD²^* and John Griffin, PhD³

¹Rabin Medical Center, Ophthalmology Department, Laboratory of Eye Research Felsenstein Medical Research Center, Petah-Tikva, Israel
²National Hemophilia Center, Sheba Medical Center, Tel Hashomer, Israel; Amalia Biron Research Institute of Thrombosis and Hemostasis, Tel Aviv University, Tel Aviv, Israel
³The Scripps Research Institute, Department of Molecular Medicine, La Jolla, CA
⁴University of Iowa, Iowa City, IA
⁵Thrombosis Unit, National Hemophilia Center, Sheba Medical Center, National Hemophilia Center, Sheba Medical Center, Tel Hashomer, Israel; Amalia Biron Research Institute of Thrombosis and Hemostasis, Tel Aviv University, New York, NY

2603

Contribution of the Residues 488-490 on the Factor (F)VIII A2 Domain and the Residue 2239 on the FVIII C2 Domain for the Interaction between FVIII and Protein S

Shoko Furukawa, MD, PhD¹^*, Masahiro Takeyama¹ and Keiji Nogami²^*

¹Pediatrics, Nara Medical University, Kashihara, Japan
²Nara Medical University, Nara, Japan

2604

Functional Assessment of VWD Type 2N Variants Using Mammalian Cell Displayed Surface-Tethered Von Willebrand Factor

Kerry W Lannert, MT(ASCP)^1,2^*, Shelley N Fletcher³^*, Nicholas A Popp⁴, Pamela A Christopherson⁵^*, Daniel J Hampshire⁶^*, Doug M Fowler^4,7^*, Andrew Yee⁸^* and Jill M Johnsen, MD^1,2

¹Institute for Stem Cell and Regenerative Medicine, University of Washington, Seattle, WA
²Department of Medicine, University of Washington, Seattle, WA
³Bloodworks, Seattle, WA
⁴Department of Genome Sciences, University of Washington, Seattle, WA
⁵Versiti Blood Center of Wisconsin, Milwaukee, WI
⁶Centre for Biomedicine, Hull York Medical School, Hull, GBR
⁷Department of BioEngineering, University of Washington, USA, Seattle, WA
⁸Department of Pediatrics, Baylor College of Medicine, Houston, TX

2605

In Vitro Analysis of Bone Remodeling Alterations in Hemophilia: Influence of Coagulation Factors on Bone Cells

Stefano Lancellotti¹^*, Giulia Battafarano²^*, Monica Sacco³^*, Maira Tardugno³^*, Leonardo Di Gennaro, MD¹^*, Matteo Luciani⁴^*, Raimondo De Cristofaro, MD, PhD^1,3^* and Andrea Del Fattore²^*

¹Fondazione Policlinico Gemelli IRCCS, Roma, Italy
²Bambino Gesù Children's Hospital, IRCCS, Rome, ITA
³Catholic University of the Sacred Heart, Rome, Italy
⁴Bambino Gesù Children's Hospital, IRCCS, Roma, Italy

2606

Characterization and Quantification of Von Willebrand Factor Proteoforms in Von Willebrand Disease Patients and Healthy Controls: Insights from the Win-Study

Iris charlotte Kreft¹^*, Tirsa T van Duijl²^*, Calvin Van Kwawegen³^*, Winny Phan¹^*, Margo Schuller⁴^*, Carmen van Der Zwaan²^*, Alexander Meijer, PhD⁴^*, Ruben Bierings⁵^*, Frank W.G. Leebeek, MD, PhD³ and Maartje Van Den Biggelaar⁴^*

¹Sanquin Research, Amsterdam, Netherlands
²Sanquin Research, amsterdam, Netherlands
³Department of Haematology, Erasmus University Medical Center-Erasmus MC, Rotterdam, Rotterdam, Netherlands
⁴Sanquin Research, Amsterdam, NLD
⁵Erasmusmc, University Medical Center Rotterdam, Rotterdam, NLD

2607

Mass Spectrometry Based Detection of Naturally Expressed FVIII in Liver Sinusoidal-like Endothelial Cells from Healthy Donor and HA Patients with Nonsense Mutations

Payal Chawla¹^*, Melanie Rath²^*, Johannes Oldenburg, MD³ and Heike Singer⁴^*

¹Institute of Experimental Hematology and Transfusion medicine, University Clinic Bonn, Bonn, Germany
²Ukb, Bonn, NW, Germany
³Institute of Experimental Haematology and Transfusion Medicine, University Clinic Bonn AöR, Medical Faculty, Bonn, Germany
⁴Ukb, Bonn, Germany

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^*signifies non-member of ASH