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111.O2.6 SCD Model Systems

Monday, December 8, 2008: 1:30 PM-3:00 PM
2002-2004 - West (Moscone Center)
Moderators:
Susan B. Shurin, MD and Andrew D. Campbell, MD
1:30 PM
Effects of Pan-Selectin Antagonist GMI-1070 on the Treatment of Vaso-Occlusion in Sickle Cell Mice

Jungshan Chang1*, John T Patton2*, Paul S. Frenette1 and John L. Magnani2*

1Department of Medicine and Immunology Institute, Mount Sinai School of Medicine, New York, NY
2GlycoMimetics, Inc, Gaithersburg, MD

1:45 PM
Pomalidomide Augments Erythropoiesis and Fetal Hemoglobin Production in a Humanized Mouse Model of Sickle Cell Disease

Steffen E. Meiler1, Marlene Wade1*, Zhong Chen1*, Preetha Ramalingam2*, Laure A Moutouh-de Parseval3*, Laura G Corral4, Ferdane Kutlar5* and Abdullah Kutlar5*

1Anesthesiology & Periop. Med., Medical College of Georgia, Augusta, GA
2Pathology, Medical College of Georgia, Augusta, GA
3Celgene Corporation, Summit, NJ
4Celgene Corporation, San Diego, CA
5Sickle Cell Center, Medical College of Georgia, Augusta, GA

2:00 PM
huGTPCH Expressing S+S-Antilles Mice Have Reduced Gardos Channel Activity, Endothelin-1 Expression, and Endothelin Receptor A and B Expression, While Total Biopterin and Percent Tetrahydrobiopterin Are Increased

José R. Romero, PhD1*, Adriana Muniz, PhD2*, Ashley Hale, BSc3*, Nicholas J. Alp, PhD, DM, MRCP4*, Alicia Rivera, PhD5, Sandra M. Suzuka, MS2*, Keith M. Channon, MD, FRCP4* and Mary E. Fabry, PhD2

1Division of Endocrinology, Diabetes and Hypertension, Brigham and Women's Hospital/Harvard Medical School, Boston, MA
2Hematology, Albert Einstein College of Medicine, Bronx, NY
3Department of Cardiovascular Medicine, Wellcome Trust Centre for Human Genetics, Oxford, United Kingdom
4Department of Cardiovascular Medicine, University of Oxford, Oxford, United Kingdom
5Children's Hospital Boston, Boston, MA

2:15 PM
The Protein C Pathway in Human and Murine Sickle Cell Disease: Alterations in Protein C, Thrombomodulin (TM), and Endothelial Protein C Receptor (EPCR) at Baseline and during Acute Vaso-Occlusion

Yihe Guo, PhD*, Teresa Uy, MD*, Nancy Wandersee, PhD, J. Paul Scott, MD, Hartmut Weiler, PhD, Sandra Holzhauer*, Dawn Retherford*, Thomas Foster* and Cheryl Hillery, MD

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

2:30 PM
Efficient Erythroid Differentiation of a PGD-Derived Human Pluripotent Stem Cell Line Affected with Sickle Cell Hemoglobinopathy

Marina Pryzhkova, Ph.D.1*, Xuan Yuan, MD1*, Abhai Tripathi, PhD2*, David Sullivan, MD2* and Elias Zambidis, MD, PhD1

1Institute of Cell Engineering, Stem Cell Biology Program, Johns Hopkins School of Medicine, Baltimore, MD
2Malaria Research Institute, Johns Hopkins University School of Public Health, Baltimore, MD

2:45 PM
High Resolution Tertiary and Quaternary Structural Changes in Deoxy and Liganded Hemoglobin E

Rhoda Elison Hirsch, PhD, Vladimir Malashkevich*, Tatiana C. Balazs* and Steven C. Almo*

Albert Einstein College of Med., Bronx, NY

*signifies non-member of ASH