Hemoglobin and Its Diseases

Subject Area(s): Human Biology and Disease; Biochemistry

Edited by David Weatherall, Weatherall Institute of Molecular Medicine; Alan N. Schechter, National Institutes of Health; David G. Nathan, Dana-Farber Cancer Institute

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Description

Hemoglobin is an iron-containing protein in red blood cells that transports oxygen to tissues throughout the body. The abundance, stability, and oxygen-carrying properties of hemoglobin can be altered by genetic mutations. More than 1000 hemoglobin disorders are known; hemoglobinopathies (e.g., sickle cell disease) and thalassemias are some of the most common human genetic diseases worldwide.

Written and edited by experts in the field, this collection from Cold Spring Harbor Perspectives in Biology covers all aspects of hemoglobin and its diseases. Contributors examine the structure, expression, and evolution of the globin genes, the assembly of globin subunits into functional forms of hemoglobin, and the numerous variants that result from genetic alterations. The pathophysiological consequences of hemoglobin disorders (e.g., ineffective erythropoiesis and aberrant iron homeostasis), their clinical manifestations, and epidemiological trends are also described.

This book includes discussions of management and treatment strategies for hemoglobin disorders, such as transfusions, iron-chelating agents, gene therapy, and stem cell transplantation. It is an indispensable reference for biochemists, geneticists, cell and developmental biologists, physiologists, and all who are interested in reducing the medical burden of these common genetic diseases.

Preface

Hemoglobin and its Disorders: 150 Years of Study: David J. Weatherall, Alan N. Schechter, and David G. Nathan
Erythopoiesis: Development and Differentiation: Elaine Dzierzak and Sjaak Philipsen
Erythropoietin: H. Franklin Bunn
Evolution of Hemoglobin and Its Genes: Ross C. Hardison
Transcriptional Mechanisms Underlying Hemoglobin Synthesis: Koichi R. Katsumura, Andrew W. DeVilbiss, Nathaniel J. Pope, Kirby D. Johnson, and Emery H. Bresnick
The Switch from Fetal to Adult Hemoglobin: Vijay G. Sankaran and Stuart H. Orkin
Iron Metabolism: Interactions with Normal and Disordered Erythropoiesis: Tomas Ganz and Elizabeta Nemeth
Erythroid Heme Biosynthesis and Its Disorders: Harry A. Dailey and Peter N. Meissner
Classification of the Disorders of Hemoglobin: Bernard G. Forget and H. Franklin Bunn
World Distribution, Population Genetics, and Health Burden of the Hemoglobinopathies: Thomas N. Williams and David J. Weatherall
The Molecular Basis of β-Thalassemia: Swee Lay Thein
The Molecular Basis of α-Thalassemia: Douglas R. Higgs
Pathophysiology and Clinical Manifestations of the β Thalassemias: Arthur W. Nienhuis and David G. Nathan
β-Thalassemia Intermedia: A Clinical Perspective: Khaled M. Musallam, Ali T. Taher, and Eliezer A. Rachmilewitz
The Hemoglobin E Thalassemias: Suthat Fucharoen and David J. Weatherall
Clinical Manifestations of α-Thalassemia: Elliott P. Vichinsky
α-Thalassemia, Mental Retardation, and Myelodysplastic Syndrome: Richard J. Gibbons
Management of the Thalassemias: Nancy F. Olivieri and Gary M. Brittenham
The Prevention of Thalassemia: Antonio Cao and Yuet Wai Kan
The Natural History of Sickle Cell Disease: Graham R. Serjeant
The Search for Genetic Modifiers of Disease Severity in the β-Hemoglobinopathies: Guillaume Lettre
Current Management of Sickle Cell Anemia: Patrick T. McGann, Alecia Nero, and Russell E. Ware
Hematopoietic Stem Cell Transplantation in Thalassemia and Sickle Cell Anemia: Guido Lucarelli, Antonella Isgrò, Pietro Sodani, and Javid Gaziev
Development of Gene Therapy for Thalassemia: Arthur W. Nienhuis and Derek A. Persons
Pluripotent Stem Cells in Research and Treatment of Hemoglobinopathies: Natasha Arora and George Q. Daley
Hemoglobin Variants: Biochemical Properties and Clinical Correlates: Christopher S. Thom, Claire F. Dickson, David A. Gell, and Mitchell J. Weiss
Cell-Free Hemoglobin and its Scavenger Proteins: New Disease Models Leading the Way to Targeted Therapies: Dominik J. Schaer and Paul W. Buehler
Iron Deficiency Anemia: A Common and Curable Disease: Jeffery L. Miller
Index

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