Biochemical and Clinical Aspects of Hemoglobin Abnormalities

Biochemical and Clinical Aspects of Hemoglobin Abnormalities

1st Edition - January 1, 1978

Write a review

  • Editor: Winslow Caughey
  • eBook ISBN: 9780323142748

Purchase options

Purchase options
DRM-free (PDF)
Sales tax will be calculated at check-out

Institutional Subscription

Free Global Shipping
No minimum order


Biochemical and Clinical Aspects of Hemoglobin Abnormalities contains the proceedings of a symposium held on the Pingree Park campus of Colorado State University on October 2-7, 1977. Contributors discuss the biochemical and clinical aspects of hemoglobin abnormalities and cover topics ranging from amino acid substitutions to sickle cell disease, glycosylated hemoglobins, cystamine inhibition of sickling, and gelation of sickle cell hemoglobin. This volume is organized into 52 chapters and begins with a discussion of the role of distal residues in structure, ligand binding, and oxidation of hemoglobins A, Zurich, and Sydney. It then turns to functional abnormalities of whole blood in sickle cell anemia, inhibition of sickle hemoglobin gelation by amino acids and peptides, and intermolecular interactions in crystals of human deoxy hemoglobins A, C, F, and S. The chapters that follow focus on glycosylation of human hemoglobin, the phase transitions of sickle-cell hemoglobin, conformational effects of the HbS mutation, and mechanisms for hemoglobin oxidation. The reader is also introduced to oxidation of oxyhemoglobin by reductants, the kinetics of oxygen binding to human red blood cells, and oxidation of human hemoglobin by copper. A chapter that assesses the effect of physiological parameters, such as pH, oxygen concentration, protein concentration, non-gelling hemoglobins, and the erythrocyte membrane, on the kinetics of polymerization of deoxyhemoglobin S concludes the book. This book is intended for biochemists and clinicians interested in knowing more about hemoglobin abnormalities.

Table of Contents

  • List of Contributors


    Role of Distal Residues in Structure, Ligand Binding, and Oxidation of Hemoglobins A, Zurich, and Sydney

    Properties and Reaction Mechanism of Hb Zurich (α2β2→HIS(63) → ARG)

    Ligand Binding to Hemoglobins: Effects of Globin Structure

    Functional Abnormalities of Whole Blood in Sickle Cell Anemia

    Hemostatic Alterations in Sickle Cell Anemia

    Assessment of the Clinical Severity of Sickle Cell Disease

    Location of the Heme Iron Atoms and Characterization of the Quaternary Structure of the Carbonmonoxy-β4 Tetramer

    Hemoglobin Engineering: Consequences of Alterations at Functionally Sensitive Sites Particularly Susceptible to Chemical or Enzymatic Attack

    The Oxygen Binding in Abnormal Cobalt Myoglobins and Hemoglobins (Discussion only)

    Amino Acids and Peptides as Inhibitors of Sickle Hemoglobin Gelation

    Effect of DBA on Hemoglobin SS Cells and Hemoglobin Biosynthesis

    Progress in the Natural History Studies of the Clinical Severity of Sickle Cell Disease: Epidemiologic Aspects

    Intermolecular Interactions in Crystals of Human Deoxy Hemoglobins A, C, F, and S

    Proton Nuclear Magnetic Resonance Studies of Sickle Cell Hemoglobin

    Areas of Interaction in the HbS Polymer

    Glycosylated Hemoglobins

    Glycosylation of Human Hemoglobin

    Evaluation of the Pool of a Hemoglobin Chains and Study of the Dissociation of the Hemoglobin Molecule into Monomers

    Inhibition of Sickling by Cystamine

    Progress in Interpreting the Phase Transitions of Sickle-Cell Hemoglobin

    Circular Dichroism Probes of Hemoglobin Structure

    Conformational Effects of the HbS Mutation

    Electron Microscopy of Fibers and Crystals of a Deoxygenated Platinum Derivative of Hemoglobin S

    Optical Detection of Heme Ligand Configuration in Sperm Whale Myoglobin

    The Role of Spectrin and Actin in Irreversibly Sickled Cells: Unsickling of "Irreversibly" Sickled Ghosts by Conditions Which Interfere with Spectrin-Actin Polymerization

    Spectrin Assembly in Irreversibly Sickled Cell Membranes: Role of Calcium and ATP

    Hemoglobin Interactions and Whole Blood Oxygen Equilibrium Curves in Sickling Disorders

    Determination of the Structure of the Fibers of Hemoglobin S by Electron Microscopy and Three-Dimensional Image Reconstruction

    Functional Identity of Hemoglobins S and A in the Absence of Polymerization

    The Apparent Absence of a Ligand-Linked Structural Transition in the Region of the ß6 Valine of Hemoglobin S

    Ligand Binding and the Gelation of Sickle Cell Hemoglobin

    Successes and Failures of a Simple Nucleation Theory for Sickle Cell Hemoglobin Gelation

    Redox System: iron(II)-iron(III) Interconversion in Oxygen-Carrying Proteins

    Mechanisms for Hemoglobin Oxidation: The Response of Abnormal Human and Other Hemoglobins to Different Oxidative Pathways

    Oxidation of Oxyhemoglobin by Reductants

    Crossings Over versus Point Mutations as Causes of Hemoglobin Variants and Possibly of Thalassemias

    Hemoglobin and the Red Cell Membrane

    Structural Aspects of Hemoglobin Function

    Effects of Heme Iron Ligands on Self-Association of ßSH Chains

    Allosteric Binding Heat Effects of HbA and HbM Iwate

    Analysis of Oxygen Equilibria in Synthetic and Natural Mutant Valence Hybrid Hemoglobins: Implications for Models of Heme-Heme Interaction in Normal Hemoglobin

    The Kinetics of Oxygen Binding to Human Red Blood Cells

    Measurement of Homeostatic Responses to Altered P50 in Patients with Abnormal Hemoglobins

    Oxidation of Human Hemoglobin by Copper: Specificity for Beta Chains and Formation of Modified Precursor

    Dichloromethane as an Antisickling Agent in Sickle Cell Hemoglobin

    Fourier Transform Infrared Spectroscopy of Hemoglobin

    A Comparative EPR Study of Hemoglobins A and Kansas: Spectral Signatures of Affinity States

    Ionic and Non-Ionic Effects on the Solubility of Deoxyhemoglobin S

    Anionic Control of Hemoglobin Function

    Inhibition of Erythrocyte Sickling in Vitro by Glyceraldehyde

    Major Sites for the Oxygen-Linked Binding of Chloride to Hemoglobin

    Effectors of the Rate of Deoxyhemoglobin S Polymerization


Product details

  • No. of pages: 746
  • Language: English
  • Copyright: © Academic Press 1978
  • Published: January 1, 1978
  • Imprint: Academic Press
  • eBook ISBN: 9780323142748

About the Editor

Winslow Caughey

Ratings and Reviews

Write a review

There are currently no reviews for "Biochemical and Clinical Aspects of Hemoglobin Abnormalities"