57th ASH Annual Meeting and Exposition (December 5-8, 2015): 113. Hemoglobinopathies, Excluding Thalassemia – Basic and Translational Science: Novel Mechanisms of Pathogenesis in Sickle Cell Disease

Hemoglobinopathies, Excluding Thalassemia – Basic and Translational Science Oral and Poster Abstracts
Oral

W340, Level 3 (Orange County Convention Center)

Moderators:

Vivien A. Sheehan, MD, PhD, Baylor College of Medicine and Carlo Brugnara, Boston Children's Hospital

Disclosures:

Brugnara: Global Blood Therapeutics: Other: Attended one Advisory Panel on 9/28/2015 .

7:00 AM

A Candidate Trans-Acting Modulator of Fetal Hemoglobin Gene Expression in the Arab-Indian Haplotype of Sickle Cell Anemia

Vinod Vathipadiekal, PhD¹^*, John Farrell, PhD²^*, Zhang Shuai³^*, Heather Edward¹^*, Heather Shappell³^*, A.M Al-Rubaish⁴^*, Fahad Al-Muhanna⁴^*, Z Naserullah⁵^*, A Alsuliman⁶^*, Irene Simkin¹^*, Lindsay Farrer⁷^*, Zhihua Jiang, PhD⁸^*, Hong Yuan Luo, PhD⁷, Shengwen Huang, PhD¹^*, Gustavo Mostoslavsky⁹^*, George J. Murphy, Ph.D¹⁰, P.K. Patra¹¹^*, David H.K. Chui, MD¹², Abdulrahman Alsultan, MD¹³, Amein Al-Ali⁴^*, Paola Sebastiani, PhD¹⁴^* and Martin H. Steinberg, MD¹

¹Boston University School of Medicine, Boston, MA
²Boston University Medical School, Boston, MA
³Boston University School of Public Health, Boston, MA
⁴University of Dammam, Dammam, Saudi Arabia
⁵King Faisal University & Departments of Pediatrics, Dammam, Saudi Arabia
⁶King Faisal University, Al-Ahsa, Saudi Arabia
⁷Boston University School of Medicine, Boston
⁸BOSTON UNIVERSITY, Boston, MA
⁹Department of Medicine, Center for Regenerative Medicine (CReM), Boston University School of Medicine, Boston, MA
¹⁰Division Of Hematology and Oncology, Center for Regenerative Medicine (CReM), Boston University School of Medicine, Boston, MA
¹¹Pt. J.N.M. Medical College, Raipur, India
¹²School of Medicine, Boston University, Boston, MA
¹³Department of Pediatrics and Sickle Cell Disease Research Center, College of Medicine, King Saud University, Riyadh, Saudi Arabia
¹⁴Department of Biostatistics, Boston University, Boston, MA

7:15 AM

410

Dimethyl Fumarate Induces Fetal Hemoglobin in Sickle Cell Disease

Sriram Krishnamoorthy, PhD¹^*, Dipti Gupta, MS¹^*, Sarah Sturtevant¹^*, Biaoru Li, PhD²^*, Levi C Makala, DVM, MBA, PhD², William E. Hobbs, MD/PhD¹, David R. Light, PhD¹ and Betty Pace, MD³

¹Hematology, Biogen, Cambridge, MA
²Department of Pediatrics and Cancer Center, Georgia Regents University, Augusta, GA
³Sickle Cell Center, Georgia Regents University, Augusta, GA

7:30 AM

411

Keap1-Nrf2 System: Potential Role in Prevention of Sickle Cell Disease Organs Damages and Inflammation

Nadine Keleku-Lukwete, MD¹^*, Mikiko Suzuki, PhD²^*, Akihito Otsuki, DDS¹^*, Kouhei Tsuchida¹^*, Saori Katayama, MD¹^*, Makiko Hayashi¹^*, Eriko Naganuma¹^*, Takashi Moriguchi, MD, PhD¹^*, Osamu Tanabe, MD, PhD³^*, James Doug Engel, PhD⁴, Masue Imaizumi, MD, PhD⁵ and Masayuki Yamamoto, MD, PhD¹

¹Department of Medical Biochemistry, Tohoku University Graduate school of Medecine, Sendai, Japan
²Center for Radioisotope Sciences, Tohoku University Graduate school of Medecine, Sendai, Japan
³Department of Integral Genomics, Tohoku Medical Megabank Organization, Sendai, Japan
⁴Department of cell and Developmental Biology, University of Michigan, Ann Arbor, MI
⁵Department of Hematology and Oncology, Miyagi Children's Hospital, Sendai, Japan

7:45 AM

412

Hemopexin Gene Therapy Inhibits Inflammation and Vaso-Occlusion in Transgenic Sickle Cell Mice

Gregory M Vercellotti¹, Ping Zhang, PhD¹^*, Chunsheng Chen, PhD¹^*, Julia Nguyen, BS¹^*, Fuad Abdulla, DVM¹^*, Phong Nguyen, BS¹^*, Carlos Nowotny¹^*, Aman Irfanullah¹^*, Ann Smith, PhD²^*, Clifford J. Steer, MD³^* and John D. Belcher, PhD¹

¹Department of Medicine, Vascular Biology Center, Division of Hematology, Oncology and Transplantation, University of Minnesota, Minneapolis, MN
²Division of Molecular Biology & Biochemistry, School of Biological Sciences, University of Missouri-K.C., Kansas City, MO
³Department of Medicine, Division of Gastroenterology, Hepatology and Nutrition, University of Minnesota, Minneapolis, MN

8:00 AM

413

Hypoxia Reoxygenation Treatment Induces Platelet Hyperactivity and Relieves Calpain-1-Mediated Inhibition of Platelet Aggregation in a Mouse Model of Severe Sickle Cell Disease

Jennifer O. Nwankwo, BS¹, Rod R. Warburton, BA²^*, Thomas Gremmel, MD³^*, Anja J. Gerrits, PhD³^*, Lauren J. Richey, DVM, PhD⁴^*, Nicholas S. Hill, MD²^*, Alicia Rivera⁵, Joseph A. Jakubowski, PhD⁶, Andrew L. Frelinger III, PhD³ and Athar H. Chishti, PhD⁷

¹Program in Pharmacology & Experimental Therapeutics, Tufts University Sackler School of Graduate Biomedical Sciences, Boston, MA
²Division of Pulmonary, Critical Care and Sleep Medicine, Tufts Medical Center, Boston, MA
³Center for Platelet Research Studies, Division of Hematology/Oncology, Boston Children's Hospital, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA
⁴Division of Laboratory Animal Medicine, Tufts University, Boston, MA
⁵Department of Laboratory Medicine, Boston Childen's Hospital and Department of Pathology, Harvard Medical School, Boston, MA
⁶Lilly Research Laboratories, Eli Lilly and Company, Indianapolis, IN
⁷Program in Pharmacology & Experimental Therapeutics, Department of Developmental, Molecular, and Chemical Biology, Tufts University School of Medicine, Boston, MA

8:15 AM

414

Platelet Nucleation on Arrested Neutrophils Drives Vaso-Occlusion in Sickle Cell Disease

Maritza A. Jimenez, B.S.^1,2^*, Prithu Sundd, PhD^2,3, Enrico M Novelli, MD, MS¹ and Gregory J Kato, MD¹^*

¹Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA
²Department of Bioengineering, University of Pittsburgh, Pittsburgh, PA
³Heart, Lung and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA

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^*signifies non-member of ASH

113.O2.6 113. Hemoglobinopathies, Excluding Thalassemia – Basic and Translational Science: Novel Mechanisms of Pathogenesis in Sickle Cell Disease