66th ASH Annual Meeting: 113. Sickle Cell Disease, Sickle Cell Trait, and Other Hemoglobinopathies, Excluding Thalassemias: Basic and Translational: Poster III

Symposia: Sickle Cell Disease, Sickle Cell Trait, and Other Hemoglobinopathies, Excluding Thalassemias: Basic and Translational Oral and Poster Abstracts
Poster

Research, Bleeding disorders, Sickle Cell Disease, Fundamental Science, Adult, Hemophilia, Sickle Cell Trait, Drug development, Translational Research, Epidemiology, Genomics, Platelet disorders, Thalassemia, Clinical Research, Hemoglobinopathies, Hematopoiesis, Thromboembolism, Diseases, Immune mechanism, Thrombocytopenias, Therapy sequence, Treatment Considerations, Immunology, Thrombotic disorders, Non-Biological therapies, Metabolism, VWD, Biological Processes, Pharmacology, Technology and Procedures, Molecular biology, Study Population, Multi-systemic interactions, Human, Pathogenesis, Animal model, Omics technologies

Halls G-H (San Diego Convention Center)

3861

Inhibition of Purine Nucleoside Phosphorylase Is a Promising Anti-Sickling Approach for Sickle Cell Disease

Adekunle Emmanuel Alagbe, MD, PhD¹, Lynda Little-Ihrig, BS²^*, Brian J Philips, PhD^3,4^*, Stephanie M Mutchler, Ph.D⁵^*, Julia Z Xu, MD, MS⁶, Edwin K Jackson, Ph.D⁷^*, Stevan Tofovic, MD, Ph.D⁷^* and Enrico M Novelli, MD, MS²

¹Pittsburgh Heart, Lung, Blood and Vascular Medicine Institute, and Division of Classical Hematology, University of Pittsburgh, Pittsburgh, PA
²Pittsburgh Heart, Lung, Blood and Vascular Medicine Institute, and Division of Classical Hematology, University of Pittsburgh, Pittsburgh, PA
³Pittsburgh Heart, Lung, and Blood Vascular Medicine Institute, Department of Medicine, University of Pittsburgh, Pittsburgh, PA
⁴Vascular Medicine Institute, Department of Medicine, University of Pittsburgh, Pittsburgh, PA
⁵Department of Medicine, Division of Renal-Electrolyte, University of Pittsburgh, Pittssburgh, PA
⁶Sickle Cell Branch, National Heart, Lung, and Blood Institute, NIH, Pittsburgh, PA
⁷Department of Pharmacology and Chemical Biology,, University of Pittsburgh, Pittsburgh, PA

3862

Transcriptional Control of Gene Expression By O-Glcnacylation during Erythropoiesis

Kenneth R Peterson, PhD¹, Matthew P Parker, PhD²^*, Aspin Denson, BS²^*, Will Brautman, BS²^*, Nick Lowe, BS²^*, Halyna Fedosyuk, MS²^*, Lesya V Novikova, PhD²^*, Jeffrey A Thompson, PhD²^*, Jennifer J Kohler, PhD³^* and Chad Slawson, PhD¹^*

¹Department of Biochemistry and Molecular Biology, University of Kansas Medical Center, Kansas City, KS
²University of Kansas Medical Center, Kansas City, KS
³University of Texas Southwestern Medical Center, Dallas, TX

3863

Pharmacological Inhibition of TMPRSS6 Decreases Hemoglobin Concentration and Red Blood Cell (RBC) Hemolysis in a Mouse Model of Sickle Cell Disease

Silvia Giannini, PhD¹, Earl Fields Jr., MPH²^*, Jesse Bueno²^*, Christopher Chambliss, PhD², Min Wu, MD, PhD¹ and David R Archer, PhD²

¹Disc Medicine, Watertown, MA
²Aflac Cancer and Blood Disorders Center, Emory University, Atlanta, GA

3864

Erythrocytes from Sickle Cell Mice Activate the Endothelium and Blood Cells and Induce Vaso-Occlusion When Transfused into ADAMTS13-Deficient Mice: Role of Lipid Degradation

Reheman Adili, MD¹, Junmei Chen, PhD¹, Nicole Roads¹^*, Richard Maldonado¹^*, Hallie Lazaro¹^*, Dominic W. Chung, Ph.D^1,2^*, Xiaoyun Fu, PhD^1,2 and José A. López, M.D^1,2

¹Bloodworks Research Institute, Seattle, WA
²University of Washington, Seattle, WA

3865

Epigenetic Clocks and Accelerated Aging in Sickle Cell Disease

Keesha Roach, PhD, RN^1,2,3, Cole Collins, B.S.⁴^*, Erielle V. Culp, B.S.¹^*, Ugochi Olivia Ogu, MD⁵ and Khyobeni Mozhui, PhD⁶^*

¹Department of Genetics, Genomics, and Informatics, University of Tennessee Health Science Center, Memphis, TN
²Department of Community and Population Health, University of Tennessee Health Science Center, Memphis, TN
³Center for Sickle Cell Disease, University of Tennessee Health Science Center, Memphis, TN
⁴College of Medicine, University of Tennessee Health Science Center, Memphis, TN
⁵Center for Sickle Cell Disease, University of Tennessee Health Sicence Center, Arlington, TN
⁶Department of Preventive Medicine, University of Tennessee Health Science Center, Memphis, TN

3866

Oral Administration of Nitrous Oxide Reduces Acute Vaso-Occlusive Pain in Mice with Sickle Cell Disease

Ana Alves, BS¹^*, Fuad Abdulla, MS²^*, Kaje Allen, BS¹^*, Iryna A. Khasabova, PhD³^*, Sergey G. Khasabov, MD, PhD³^*, Mark Young, PhD⁴^*, Andrew Gomperts, MBA, JD⁴^*, Edward Gomperts, MD⁵, John D. Belcher, PhD², Gregory M. Vercellotti, MD² and Donald A. Simone, PhD³^*

¹School of Denistry, Department of Diagnostic and Biological Sciences, University of Minnesota, Minneapolis, MN
²Division of Hematology, Oncology and Transplantation, Department of Medicine, University of Minnesota, Minneapolis, MN
³School of Dentistry, Department of Diagnostic and Biological Sciences, University of Minnesota, Minneapolis, MN
⁴Hillhurst Biopharmaceuticals, Inc, San Diego, CA
⁵Hillhurst Biopharmaceuticals, Inc, Montrose, CA

3867

Single-Cell Transcriptomic Analysis of Bone Marrow from Humanized Sickle Cell Mice Reveals a Subpopulation of Highly-Expressing S100A8/A9 Mesenchymal Stem Cells

Jacopo Ceolan¹^*, Petros Kolovos²^*, Enrica Federti³^*, Panagiotis Liakopoulos⁴^*, Veronica Riccardi⁵^*, Immacolata Andolfo, PhD⁶^*, Alessandro Mattè, BSc, PhD⁷^*, Roberta Russo⁸^*, Achille Iolascon, MD, PhD⁹^*, Helene Gleitz, PhD¹⁰^*, Sjaak Philipsen¹¹^* and Lucia De Franceschi¹²

¹Azienda Ospedaliera Universitaria di Verona, Verona, Italy
²Department of Molecular Biology & Genetics,, Democritus University of Thrace, Alexandroupolis, GRC
³Department of Engineering for Innovation Medicine, University of Verona, Verona, Italy
⁴Department of Molecular Biology & Genetics, Democritus University of Thrace, Alexandroupolis, GRC
⁵Dept of Medicine,, University of Verona and Azienda Ospedaliera Universitaria Int, Verona, Italy
⁶Department of Molecular Medicine and Medical Biotechnology, University of Naples Federico II, Naples, Italy
⁷University of Verona 'G.B. Rossi' Hospital, Verona, Italy
⁸CEINGE - Biotecnologie Avanzate Franco Salvatore, CEINGE - Biotecnologie Avanzate, Napoli, NA, Italy
⁹University of Naples Federico II, CEINGE Biotecnologie Avanzate, Naples, Italy
¹⁰Department of Developmental Biology, Erasmus Medical Center, Rotterdam, NLD
¹¹Erasmus University Medical Center, Rotterdam, NLD
¹²University of Verona, Verona, Italy

3868

Novel Selective HDAC1/2 Inhibitor 2-Aminobenzamide Derivative Potently Induces Fetal Hemoglobin

Victor Gabriel de Faria Pastre, BSc¹^*, Aline Renata Pavan, PhD²^*, Carolina Lanaro, BSc, PhD²^*, Dulcineia Albuquerque, PhD²^*, Rafael Consolin Chelucci, PhD³^*, Leonardo Luiz Gomes Ferreira, PhD³^*, Adriano D. Andricopulo, PhD³^*, Jean Leandro Dos Santos, PhD⁴^* and Fernando Ferreira Costa, MD, PhD⁵

¹Hematology and Hemotherapy Center, University of Campinas - UNICAMP, Campinas, SP, Brazil
²Hematology and Hemotherapy Center, University of Campinas - UNICAMP, Campinas, Brazil
³Physics Institute of Sao Carlos, University of Sao Paulo, Sao Carlos, Brazil
⁴School of Pharmaceutical Sciences, Sao Paulo State University, Araraquara, Brazil
⁵Hematology and Hemotherapy Center, University of Campinas, Campinas, Brazil

3869

Mechanisms Underlying Acute Pain in Sickle Cell Disease

Carolina Mireles, BS¹^*, Hemanth M Cherukury, PhD^1,2, Yugal Goel³^*, Kendall O'Daniel, BS¹^*, Mya Arellano, BS¹^*, Donovan Alexander Argueta, PhD¹ and Kalpna Gupta, PhD^1,2,4

¹Hematology/Oncology Division, Department of Medicine, University of California, Irvine, Irvine, CA
²Department of Pharmaceutical Sciences, University of California, Irvine, Irvine
³Hematology/Oncology Division, Department of Medicine, University of California, Irvine, Orange, CA
⁴Division of Hematology, Oncology and Transplantation, Department of Medicine, University of Minnesota, Minneapolis, MN

3870

Complement Activation and Its Implication in the Pathophysiology of Hemolytic Anemia and Aging in Mouse Models of Sickle Cell Disease and Beta-Thalassemia

Thiago Trovati Maciel, PhD¹^*, Nabiha Sbeih, MD¹^*, Rachel Rignault-Bricard¹^*, Isabelle Chevalme¹^*, Slimane Allali^1,2^*, Camille Brochier, PhD³^*, Anna Kiialainen⁴^*, Tovo David⁴ and Olivier Hermine, MD, PhD^1,5

¹Institut Imagine - INSERM U1163, Paris, France
²Sickle Cell Center, Pediatrics Department, Necker Hospital for Sick Children, APHP, Paris Cité University, Paris, FRA
³Institut Roche, Boulogne Billancourt, France
⁴F. Hoffmann-La Roche Ltd, Basel, Switzerland
⁵Hematology, Necker Hospital, Greater Paris University Hospitals (AP-HP), Paris, France

3871

Mechanism-Based Treatable Targets for Organ Damage and Pain in Sickle Cell Disease

Yugal Goel¹^*, Sirsendu Jana, PhD²^*, Mya Arellano, BS³^*, Reina A Lomeli³^*, Graham J Velasco, MSHCA⁴^*, Donovan A Argueta, PhD³, Richard Prince, PhD⁵^*, Abdu I Alayash, PhD²^*, Joel Friedman, PhD^5,6^* and Kalpna Gupta, PhD^3,7

¹Hematology/Oncology Division, Department of Medicine, University of California, Irvine, Orange, CA
²Laboratory of Biochemistry and Vascular Biology, Center for Biologics Evaluation and Research, Food and Drug Administration, Silver Spring, MD
³Hematology/Oncology Division, Department of Medicine, University of California, Irvine, Irvine, CA
⁴Pathology Department, VA Long Beach Healthcare System, Long Beach, CA
⁵Vascarta Inc, Summit, NJ
⁶Department of Microbiology and Immunology, Albert Einstein College of Medicine, Bronx, NY
⁷Division of Hematology, Oncology and Transplantation, Department of Medicine, University of Minnesota, Minneapolis, MN

3872

The Association between Urinary Biomarkers 3-Indoxyl Sulfate, Monocyte Chemoattractant Protein-1, Neutrophil Gelatinase-Associated Lipocalin and Klotho with Chronic Kidney Disease in Patients with Sickle Cell Disease

Laila Elsherif, PhD¹, Yanxin Chen, BS²^*, Kammie L. Patillo, RN³^*, Paula McCune, RN³^*, Qingning Zhou, PhD⁴^* and Kenneth I. Ataga, MD¹

¹Center for Sickle Cell Disease, The University of Tennessee Health Science Center, Memphis, TN
²College of Medicine, The University of Tennessee Health Science Center, Memphis, TN
³Office of Clinical Trials, The University of Tennessee Health Science Center, Memphis, TN
⁴Department of Mathematics and Statistics, The University of North Carolina at Charlotte, Charlotte, NC

3873

The Intracellular Effects of Anti-Polymerizing RNA Aptamers in Sickled Red Blood Cells

Alicia Matthews, BS¹^*, Christopher Hu, BS¹^*, Zoe Sekyonda, BS²^*, Umut Gurkan, PhD³ and Yolanda Fortenberry, Ph.D.¹

¹Biology Department, Case Western Reserve University, Cleveland, OH
²Department of Biomedical Engineering, Case Western Reserve University, Cleveland, OH
³Case Western Reserve University, Shaker Heights, OH

3874

Low Dose Clodronate Improves Exercise Tolerance, Right Ventricular Function, and Alters Resident Lung Macrophage Phenotypes

Christina Lisk, PhD¹^*, Francesca I Cendali, BS², Delany Swindle¹^*, Gemlyn George, MD¹^*, Kathryn Louise Hassell, MD¹, Rachelle Nuss, MD³^*, Angelo D'Alessandro, PhD⁴, Paul Buehler, PharmD, PhD⁵^* and David Irwin, Ph.D¹^*

¹University of Colorado Anschutz Medical Campus, Aurora, CO
²University of Colorado School of Medicine, Anschutz, Aurora, CO
³Center for Cancer and Blood Disorders, University of Colorado Anschutz Medical Campus, Aurora, CO
⁴Department of Biochemistry and Molecular Genetics, University of Colorado Anschutz Medical Campus, Aurora, CO
⁵Center for Blood Oxygen Transport and Hemostasis, University of Maryland, Baltimore, MD

3875

Further Validation of Flow Adhesion of Whole Blood to P-Selectin (FA-WB-PSEL) to Assess Targeted P-Selectin Therapies in Sickle Cell Disease (SCD)

Jennell U. White, PhD^1,2, Xiufeng Gao, MD³^*, Kerry Bellemare⁴^*, Gershwin Theophilus Blyden, MD, PhD⁵^*, Lanetta Bronté-Hall, MD, MPH, MSPH⁶, Alexander K Glaros, MD⁷^*, Andrew D. Campbell, MD⁸, Olufunke Y. Martin, MD⁹, Sharon Pennington, MD¹⁰, Ofelia A. Alvarez, MD¹¹, Indryas L. Woldie, MD^12,13, Nnamdi Okeke, PharmD¹⁴^* and Patrick C. Hines, MD, PhD^1,15

¹Functional Fluidics Inc, Detroit, MI
²Department of Pharmacology/Pediatrics, Wayne State University, Grosse Pointe Woods, MI
³Functional Fluidics, Detroit, MI
⁴Pharmacology, Wayne State University School of Medicine, Detroit
⁵Foundation for Sickle Cell Disease Research, Miami, FL
⁶The Foundation for Sickle Cell Disease Research, Hollywood, FL
⁷Central Michigan University College of Medicine, Mount Pleasant, MI
⁸Center for Cancer and Blood Disorders, Children's National Medical Center, Washington, DC
⁹Center for Cancer and Blood Disorders, Children's Medical Center Dallas, Dallas, TX
¹⁰Mississippi Center For Advanced Medicine, Madison, MS
¹¹Department of Pediatrics, Division of Pediatric Hematology/Oncology, Univ. of Miami School of Med., Miami, FL
¹²Wayne State University, Detroit
¹³Karmanos, Detroit, MI
¹⁴Functional Fluidics, Detroit
¹⁵Department of Pediatrics, Wayne State University School of Medicine, Detroit, MI

3876

Dynamic Cerebral Autoregulation and Blood Pressure Oscillations Reveal the Severity of Autoregulatory Dysfunction in Sickle Cell Disease

Joel Dzidzorvi Kwame Disu, BSc, MSc¹^*, Lara Abdelmohsen^1,2^*, Nahom Mossazghi, BS, MS¹^*, Elizabeth Meinert-Spyker¹^*, Christine Saber, MD^3,4,5^*, Julia Xu, MD, MS^4,5,6,7 and Sossena Wood, PhD, BSc^1,8^*

¹Biomedical Engineering, Carnegie Mellon University, Pittsburgh, PA
²Carnegie Mellon University, Pittsburgh
³Pittsburgh Heart, Lung and Blood Vascular Medicine Institute, Department of Medicine, University of Pittsburgh, Pittsburgh, PA
⁴Vascular Medicine Institute, Department of Medicine, University of Pittsburgh, Pittsburgh, PA
⁵Division of Classical Hematology, Department of Medicine, University of Pittsburgh, Pittsburgh, PA
⁶University of Pittsburgh, Pittsburgh, PA
⁷Sickle Cell Branch, National Heart, Lung, and Blood Institute, NIH, Pittsburgh, PA
⁸Carnegie Mellon University, Pittsburgh, PA

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^*signifies non-member of ASH