The Molecular Basis of Mutant Hemoglobin Dysfunction

The Molecular Basis of Mutant Hemoglobin Dysfunction

1st Edition - January 1, 1981

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  • Editor: Paul B. Sigler
  • eBook ISBN: 9781483165219

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The Molecular Basis of Mutant Hemoglobin Dysfunction contains the proceedings of the Comprehensive Sickle Cell Center Symposium on the Molecular Basis of Mutant Hemoglobin Dysfunction held at the University of Chicago, Chicago, Illinois, USA, on 7-10 October 1979. The symposium aims to document the progress of research efforts and bring together biochemists, geneticists, clinicians, counselors, and administrators, so that ensuing research will bring science to bear more effectively on these diseases. The volume contains 31 chapters organized into six sections. Section I presents two papers on the influence of fetal hemoglobin on the risk of complications and ocular manifestations of sickle cell disease. The papers in Section II deal with normal and abnormal gene expression. Section IIII is devoted to the structural analysis of mutant hemoglobins and their aggregates. Section IV focuses on evaluations of the physical and molecular basis of mutant hemoglobin dysfunction. The presentations in Section V cover cell biology and pathophysiology of sickle cell disease. Section VI reviews the status of the therapy of sickle cell disease.

Table of Contents

  • Dedication


    Plenary Address: Molecular Disease

    Section I: Some Clinical Problems Stated for the Molecular Biologist

    The Influence of Fetal Hemoglobin on the Risk of Complications of Sickle Cell Anemia

    Ocular Manifestations of Sickle Cell Disease

    Section II: Expression of Normal and Abnormal Genes

    Introductory Remarks

    Organization of Normal and Abnormal Human Globin Genes by Restriction Enzyme Analysis

    The α-Globin Genotype as a Determinant of Hematologic Parameters in Sickle Cell Trait

    Characterization of Linked Human Globin Genes by Molecular Cloning Procedures

    Regulation of Human Globin Gene Expression after Gene Transfer

    The Introduction of Normal and Mutant Globin Genes into Mammalian Cells Using SV40 Vectors

    Section III: Structure Analysis of Mutant Hemoglobins and their Aggregates

    Introductory Remarks

    Flexibility of the NH2-Terminal Region of the ß Chains of Hemoglobin: Correlation with the Gelation Properties of Deoxyhemoglobin S

    Contacts Between Molecular Surfaces in Crystals of Deoxygenated Human Hemoglobins A, C, F, and S

    Double Filaments: the Basic Structural Unit of

    Deoxygenated Sickle Hemoglobin Fibers

    A Plausible Molecular Model for the 14-Filament Fibers of Sickle Cell Hemoglobin

    Polymorphic Assemblies of Double Strands of Sickle Cell Hemoglobin and their Role in Fiber and Crystal Formation

    The Effect of Additive Conformation on Enhancement of Deoxyhemoglobin Polymerization

    Section IV: Molecular Dynamics of Dysfunction and Aggregation

    Introductory Remarks

    Subunit Assembly and Interactions in Normal and Abnormal Human Hemoglobins

    Is It Possible to Deduce the Interaction Between Two Proteins from Their Three-Dimensional Structure?

    Peptide Inhibitors of the Gelation of Sickle Hemoglobin

    Oxygen Binding and the Gelation of Sickle Cell Hemoglobin

    Rheological Properties of the Gelled Phase of Hemoglobin S

    Decreased Binding of 2,3-Diphosphoglycerate to Deoxy Hemoglobin S: A Polymerization-Independent Functional Abnormality

    Section V: Cell Biology and Pathophysiology of Sickle Cell Disease

    Introductory Remarks

    Red Cell Membrane Alterations Associated with the Sickling Phenomenon

    Sickle Erythrocyte Adherence to Cultured Human Endothelial Cells

    Section VI: Prospects for Therapy at the Molecular Level

    Introductory Remarks

    Prospects for Therapy at the Molecular Level: Historical Review

    Covalent Inhibitors of Sickling

    Weak Binding Gases as Modulators of Hemoglobin Function

    Chemical Modifications of Hemoglobin S at the 2,3-Diphosphoglycerate Binding Site: An Approach to Therapy of Sickle Cell Disease


Product details

  • No. of pages: 352
  • Language: English
  • Copyright: © North Holland 1981
  • Published: January 1, 1981
  • Imprint: North Holland
  • eBook ISBN: 9781483165219

About the Editor

Paul B. Sigler

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