UID:
almahu_9948025476602882
Umfang:
1 online resource (293 p.)
ISBN:
1-281-00562-2
,
9786611005627
,
0-08-049209-6
Inhalt:
Paroxysmal Nocturnal Hemoglobinuria (PNH) has been recognized for over a century. This mysterious disease is now understood at the level of the gene and the protein. The pathophysiology is related to a class of cell surface proteins with distinctive biochemical and physical characteristics. Recently it has been acknowledged that PNH is not rare, and once sensitive assays--based on the chemistry of the proteins--can be applied to many patients.Written by international experts in the field, this book includes a number of distinctive characteristics, such as the clinical features of PNH, the
Anmerkung:
Description based upon print version of record.
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Front Cover; Paroxysmal Nocturnal Hemoglobinuria and the Glycosylphosphatidylinositol- Linked Proteins; Copyright Page; Contents; Contributors; Preface; Chapter 1. A Brief History of PNH; The Diagnosis of PNH; Summary; References; Chapter 2. Genetics of PNH; Biochemical Genetics of PNH in Somatic Cells; The PIG-A Gene; The PIG-A Protein; Detecting Mutations in the PIG-A Gene; Germ Line PIG-A Mutations; The Spectrum of Somatic Mutations in the PIG-A Gene; PIG-A Mutations without PNH; Structure-Function Relationships; Correlation between Phenotypes and Genotypes
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Comparison of the Spectrum of Mutations in Three X-Linked Genes: PIG-A, FVIII, and G6PDConclusion; References; Chapter 3. Hemolysis in PNH; Introduction; The Classical Pathway of Complement; The Alternative Pathway of Complement; Phenotypic Mosaicism; Functional Basis of the Abnormal Sensitivity of the Erythrocytes of PNH to Complement-Mediated Lysis; Treatment of Hemolysis; Summary and Conclusions; References; Chapter 4. Thrombotic Complications in PNH; Introduction; Incidence of Thrombosis in PNH; Factors Associated with Venous Thrombosis; Platelet Abnormalities in PNH
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Role of Red Cell and Platelet MicrovesiclesUrokinase Activator Receptor (uPAR) and Cell-Mediated Fibrinolysis; Treatment of Thrombosis in PNH; Summary; References; Chapter 5. Bone Marrow Failure in PNH; Introduction; Pathophysiology of Marrow Failure in PNH; An Immune Model of PNH Pathogenesis; Clinical Course and Treatment of Marrow Failure in PNH; Summary; References; Chapter 6. Animal Models of PNH; Introduction; Mouse Piga Gene; In Vitro Differentiation of PIGA(- ) ES Cells; Chimeric Mice Bearing PIGA(- ) Hematopoietic Stem Cells; Hematopoiesis by Cells from Patients with PNH in SCID Mice
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Conclusions and PerspectivesReferences; Chapter 7. The Function of GPI-Anchored Proteins; Introduction; GPI-Linked Proteins and Cell Signaling; Role of GPI-Anchored Proteins in Insulin Action; GPI-Anchored Proteins Function as Receptors; GPI Anchors and Membrane Structure; Summary; References; Chapter 8. GPI in Lower Animals; Introduction; Structures of Protozoal GPIs; Evidence for GPI-Mediated Signaling in Mammalian Cell Biology; Signal Transduction in Host Cells by Protozoal GPIs; Parasite GPIs as Functional Toxins and Pathogenicity Factors
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Regulation of Macrophage Function by Glycoconjugates of LeishmaniaRegulation of Host Cells by Ceramide-Containing GPIs of T. cruzi; Insulin-Mimetic Activities of Protozoal GPIs; CD1d-Restricted NK T Cells and Immunity to GPI-Anchored Proteins; Conclusions; References; Chapter 9. Synthesis of the GPI Anchor; Introduction; GPI Biosynthetic Pathway; Mammalian and Yeast GPI-Anchor Defective Mutants; GPI-Anchor Addition to Proteins; Biological Implications of GPI Anchoring; Conclusions; References; Chapter 10. Functional and Structural Organization of GPI-Anchored Proteins in Cellular Membranes
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Introduction
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English
Weitere Ausg.:
ISBN 0-12-772940-2
Sprache:
Englisch
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