TY - CHAP M1 - Book, Section TI - Hemoglobin S Polymerization and Sickle Cell Disease: An Overview A1 - Eaton, William A. A2 - Gladwin, Mark T. A2 - Kato, Gregory J. A2 - Novelli, Enrico M. PY - 2021 T2 - Sickle Cell Disease AB - Polymerization of hemoglobin S is the root cause of the pathology of sickle cell disease. It has been 70 years since the legendary 20th-century genius of chemistry, Linus Pauling, discovered that aggregation of an abnormal hemoglobin into “rigid rods” is responsible for sickle cell disease.1,2 Pauling left several important questions unanswered that have motivated an enormous amount of basic research since then. These include the following: What is the structure of his “rigid rods?”3 What are the thermodynamics, kinetics, and mechanisms of polymerization? How can the disease be treated? This overview will briefly describe research that has played a major role in answering these questions. A more extensive account of polymerization and its role in disease pathogenesis and therapy can be found in 4 previous reviews4-7 and in Chapter 2 of this book. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/28 UR - hemonc.mhmedical.com/content.aspx?aid=1179345960 ER -