CELL MEMBRANE STUDIES: RED BLOOD CELL MODEL
CONTENTS INTRODUCTION: HISTORY OF RED CELL MEMBRANE RESEARCH INVENTION OF OPTICAL MICROSCOPES AND THEIR APPLICATION TO HEMATOLOGY DISCOVERY OF HEREDITARY SPHEROCYTOSIS BY LIGHT MICROSCOPY THE DAWN OF RED CELL MEMBRANE RESEARCH COMMENCEMENT OF MEMBRANE PROTEIN BIOCHEMISTRY: INTRODUCTION OF SODIUM DODECYL SULFATEPOLYACRYLAMIDE GEL ELECTROPHORESIS ELUCIDATION OF THE PATHOGENESIS OF RED CELL MEMBRANE DISORDERS GENOTYPES OF RED CELL MEMBRANE DISORDERS REEVALUATION OF MOLECULAR ELECTRON MICROSCOPY FOR PHENOTYPES COMPOSITION OF NORMAL RED CELL MEMBRANES INTRODUCTION MEMBRANE LIPIDS THE CONTENTS AND NATURE OF MEMBRANE LIPIDS ASYMMETRY OF THE MEMBRANE LIPID BILAYER MEMBRANE FLUIDITY RENEWAL OF MEMBRANE LIPIDS INTERACTIONS BETWEEN MEMBRANE LIPIDS AND PROTEINS MEMBRANE LIPIDS AS A DETERMINANT OF RED CELL SHAPE MEMBRANE PROTEINS SEPARATION AND IDENTIFICATION OF MEMBRANE PROTEINS MEMBRANE PROTEINS AND MEMBRANE STRUCTURE MEMBRANE PROTEINS IN THE RED CELL SURFACE MEMBRANE PROTEINS AND MEMBRANE FUNCTIONS RED CELL MORPHOLOGY AND SHAPE CHANGE RED CELL DEFORMABILITY MEMBRANE TRANSPORT AND PERMEABILITY V CONTENTS STEREOTACTIC STRUCTURE OF RED CELL MEMBRANES HISTORICAL BACKGROUND TO MEMBRANE MODELS ULTRASTRUCTURE OF RED CELL MEMBRANES EVALUATION OF THE CYTOSKELETAL NETWORK ELECTRON MICROSCOPY WITH THE NEGATIVE STAINING METHOD ELECTRON MICROSCOPY WITH THE QUICK-FREEZE DEEP-ETCHING (QFDE) METHOD ELECTRON MICROSCOPY WITH THE SURFACE REPLICA (SR) METHOD INTEGRAL PROTEINS EXAMINED BY ELECTRON MICROSCOPY WITH THE FREEZE FRACTURE METHOD VISUALIZATION OF GLYCOPHORINS BY FIELD EMISSION SCANNING ELECTRON MICROSCOPY SKELETAL PROTEINS AND -SPECTRINS STRUCTURE OF RED CELL SPECTRINS FUNCTIONS OF RED CELL SPECTRINS ERYTHROID AND NONERYTHROID SPECTRINS BINDING TO OTHER MEMBRANE PROTEINS EXTENSIVE ALTERNATIVE SPLICINGS NONERYTHROID PROTEIN 4 1 ISOFORMS ACTIN OTHER MINOR SKELETAL PROTEINS THE P55 PROTEIN ADDUCIN DEMATIN (PROTEIN 4 9) TROPOMYOSIN TROPOMODULIN OTHER MEMBRANE PROTEINS INTEGRAL PROTEINS BAND STRUCTURE OF BAND FUNCTIONS OF BAND MEMBRANE PROTEIN BINDING BY THE CYTOPLASMIC DOMAIN OF BAND BINDING TO GLYCOLYTIC ENZYMES BY THE CYTOPLASMIC DOMAIN OF BAND BINDING TO HEMOGLOBIN BY THE CYTOPLASMIC DOMAIN OF BAND ANION EXCHANGE CHANNEL BY THE TRANSMEMBRANE DOMAIN OF BAND LATERAL AND ROTATIONAL MOBILITY OF BAND BLOOD TYPE ANTIGENS AND BAND BAND 3 IN NONERYTHYROID CELLS GLYCOPHORINS GLYCOPHORINS A, B, AND E GLYCOPHORIN A (GPA) GLYCOPHORIN B (GPB) GLYCOPHORIN E (GPE) GYLCOPHORINS C AND D GLYCOPHORIN C (GPC) GLYCOPHORIN D (GPD) BLOOD GROUP ANTIGENS ABO BLOOD GROUP RH BLOOD GROUP P BLOOD GROUP LUTHERAN BLOOD GROUP KELL BLOOD GROUP LEWIS BLOOD GROUP DUFFY BLOOD GROUP KIDD BLOOD GROUP LW BLOOD GROUP II BLOOD GROUP THE DIEGO AND WRIGHT BLOOD GROUP ANTIGENS ON BAND OTHER MINOR BLOOD GROUP ANTIGENS GLYCOSYL PHOSHATIDYLINOSITOL (GPI) ANCHOR PROTEINS ANCHORING PROTEINS ANKYRIN STRUCTURE OF RED CELL ANKYRIN MEMBRANE (BAND 3)-BINDING DOMAIN OF ANKYRIN SPECTRIN-BINDING DOMAIN OF ANKYRIN REGULATORY DOMAIN OF ANKYRIN FUNCTIONS OF ANKYRIN ERYTHROID AND NONERYTHROID ANKYRINS PROTEIN 4 2 IN RED CELL MEMBRANE ULTRASTRUCTURE PROTEIN 4 2 GENE CHARACTERISTICS OF GENOMIC DNA CDNA OF THE PROTEIN 4 2 GENE PROTEIN 4 2 GENE IN MOUSE RED CELLS TISSUE-SPECIFIC EXPRESSION OF THE MOUSE PROTEIN 4 2 GENE AND THE PALLID MUTATION MEMBRANE MORPHOGENESIS IN ERYTHROID CELLS RED CELL MEMBRANE PROTEINS DURING ERYTHROID DEVELOPMENT AND DIFFERENTIATION EXPRESSION OF MEMBRANE PROTEINS IN EARLY ERYTHROID PROGENITORS (BFU-E AND CFU-E) EXPRESSION OF MEMBRANE PROTEINS IN EARLY ERYTHROBLASTS EXPRESSION OF MEMBRANE PROTEINS IN LATE ERYTHROBLASTS EXPRESSION OF RED CELL MEMBRANE PROTEIN 4 2 AND ITS MRNA IN NORMAL HUMAN ERYTHROID MATURATION DEVELOPMENTAL EXPRESSION OF MOUSE RED CELL PROTEIN 4 2 MRNA IN ERYTHROID AND NONERYTHROID TISSUES NUMBER OF 5’-CG-3’ DINUCLEOTIDE SITES AND THEIR STATES OF METHYLATION TRANSCRIPTIONAL ACTIVITY OF EPB3 AND ELB42 IN VARIOUS HUMAN CELL TYPES METHYLATION IN EPB3, ELB42, AND SPTB PROMOTERS DURING ERYTHROID DEVELOPMENT AND DIFFERENTIATION METHYLATION IN THE DISEASE STATES DISEASE STATES OF RED CELL MEMBRANES: THEIR GENOTYPES AND PHENOTYPES INCIDENCE OF RED CELL MEMBRANE DISORDERS SCREENING PROCEDURES FOR MEMBRANE DISORDERS HEREDITARY SPHEROCYTOSIS (HS) DEFINITION AND HISTORY CLINICAL AND LABORATORY FINDINGS EPIDEMIOLOGY AND GENETICS PATHOGENESIS: AFFECTED PROTEINS AND THEIR RELATED GENE MUTATIONS COMBINED PARTIAL DEFICIENCY OF SPECTRIN AND ANKYRIN DUE TO ANKYRIN GENE MUTATIONS PARTIAL DEFICIENCY OF BAND 3 DUE TO THE BAND 3 GENE MUTATIONS PROTEIN 4 2 DEFICIENCY ISOLATED PARTIAL SPECTRIN DEFICIENCY CELLULAR PHENOTYPES: SPHEROCYTOSIS AND MEMBRANE TRANSPORT ROLE OF THE SPLEEN COMPLICATIONS THERAPY AND PROGNOSIS HEREDITARY ELLIPTOCYTOSIS (HE) DEFINITION AND EPIDEMIOLOGY OVERALL PATHOGENESIS ANALYSIS OF MEMBRANE PROTEIN ABNORMALITIES MOLECULAR ETIOLOGY HEREDITARY PYROPOIKILOCYTOSIS (HPP) SOUTHEAST ASIAN OVALOCYTOSIS (SAO) HEREDITARY STOMATOCYTOSIS HEREDITARY HYDROCYTOSIS HEREDITARY XEROCYTOSIS RHNULL DISEASE ACANTHOCYTOSIS AND ITS RELATED DISORDERS ABETALIPOPROTEINEMIA CHOREA-ACANTHOCYTOSIS MCLEOD SYNDROME SPUR CELLS AND TARGET CELLS ABNORMALITIES OF SKELETAL PROTEINS SPECTRIN SPECTRIN ABNORMALITIES SPECTRIN IN TOKYO SPECTRIN LE PUY IN YAMAGATA SPECTRIN NAGOYA CORRELATION BETWEEN GENOTYPE AND PHENOTYPE EXPRESSIONS IN -SPECTRIN ANOMALIES PROTEIN 4 1 ABNORMALITIES TOTAL DEFICIENCY OF PROTEIN 4 1: PROTEIN 4 1 () MADRID ABNORMALITIES OF INTEGRAL PROTEINS AND BLOOD GROUP ANTIGENS BAND 3 ABNORMALITIES TOTAL DEFICIENCY OF BAND HOMOZYGOUS MISSENSE MUTATION: BAND 3 FUKUOKA TOTAL DEFICIENCY OF PROTEIN 4 2 DUE TO THE BAND 3 GENE MUTATIONS: BAND 3 OKINAWA PARTIAL DEFICIENCY OF BAND 3 IN HEREDITARY SPHEROCYTOSIS GLYCOPHORIN A AND B VARIANTS GLYCOPHORIN C AND D VARIANTS RH BLOOD GROUP ANTIGENS THE KELL BLOOD GROUP ANTIGENS (THE MCLEOD SYNDROME) ABNORMALITIES OF ANCHORING PROTEINS ANKYRIN MUTATIONS IN HEREDITARY SPHEROCYTOSIS ANKYRIN MARBURG AND ANKYRIN STUTTGART 16 2 2 1 CLINICAL HEMATOLOGY 16 2 2 2 RED CELL MEMBRANE PROTEINS 16 2 2 3 RED CELL MEMBRANE LIPIDS 16 2 2 4 RED CELL DEFORMABILITY 16 2 2 5 BIOPHYSICAL CHARACTERISTICS 16 2 2 6 MEMBRANE TRANSPORT 16 2 2 7 ULTRASTRUCTURE OF RED CELL MEMBRANES IN SITU 16 2 2 8 PROTEIN 4 2 GENE MUTATIONS 16 2 2 9 BAND 3 GENE MUTATIONS 16 2 3 1 PARTIAL OR TOTAL LACK OF ONE HAPLOID SET OF MUTATED BAND 16 2 3 2 MUTATIONS IN THE CYTOPLASMIC DOMAIN OF BAND 3, WHICH CONTAINS MAJOR BINDING SITES FOR PROTEIN 4 2 PROTEIN 4 2 DOUBLETS ABNORMALITIES OF MEMBRANE LIPIDS LECITHIN: CHOLESTEROL ACYLTRANSFERASE (LCAT) DEFICIENCY LIPOPROTEIN DEFICIENCY (ACANTHOCYTOSIS) HEREDITARY HIGH RED CELL MEMBRANE PHOSPHATIDYLCHOLINE HEMOLYTIC ANEMIA (HPCHA) LIPOPROTEIN DEFICIENCY (TANGIER DISEASE) ABNORMALITIES ASSOCIATED WITH OTHER DISEASES (TARGET CELLS AND SPUR CELLS) CLOSING REMARKS