Interest in and emphasis upon different aspects of the sphingolipids have, in general, followed the biochemical developments of the day. The early inves tigators were preoccupied principally with the isolation of "pure" compounds and structural elucidation. This historical perspective is found in the discus sion presented in Chapter 1 (Section 1. 1. 2 and Table III). Still, the isolation and structural characterization of glycolipids are the basic foundation of all our knowledge of enzymology, immunology, and cell biology. Recent infor mation obtained on structure has greatly affected the…mehr
Interest in and emphasis upon different aspects of the sphingolipids have, in general, followed the biochemical developments of the day. The early inves tigators were preoccupied principally with the isolation of "pure" compounds and structural elucidation. This historical perspective is found in the discus sion presented in Chapter 1 (Section 1. 1. 2 and Table III). Still, the isolation and structural characterization of glycolipids are the basic foundation of all our knowledge of enzymology, immunology, and cell biology. Recent infor mation obtained on structure has greatly affected the interpretation of various phenomena related to glycolipids. New structures suggest a new role of gly colipids as antigens and receptors. Ten years ago, only four neutral glycolipids and two gangliosides were known in human erythrocytes. We now know structures of at least twenty additional neutral glycolipids and ten additional gangliosides in human erythrocytes that are known to be important blood group, heterophil, and autoantigens. Erythrocytes are only one example of a cell type whose glycolipid profile has been extensively studied. Our defective knowledge in immunology and cell biology may be due to incomplete un derstanding of structural chemistry. Modern methodology based on methyla tion analysis, mass spectrometry, and enzymatic degradation has supple mented classical analysis based on clorimetry. Nuclear magnetic resonance spectroscopy is still in the development stage, but will eventually replace var ious chemical analyses. However, important future studies should be directed toward elucidating the organizational structure of glycolipids in membranes.
1 Chemistry of Glycosphingolipids.- 1.1. Introduction.- 1.1.1. Classification and Nomenclature of Glycosphingolipids.- 1.1.2. Brief History of the Chemistry of Glycosphingolipids.- 1.2. Isolation of Glycosphingolipids.- 1.2.1. Extraction.- 1.2.2. Purification of Lipid Extract: Elimination of Nonlipid Contaminants.- 1.2.3. Separation of Gangliosides and Long-Chain Neutral Glycolipids.- 1.2.4. Separation of Individual Glycolipids.- 1.3. Characterization of Glycosphingolipids.- 1.3.1. Analysis by Thin-Layer Chromatography.- 1.3.2. Characterization of Ceramide Structure, Fatty Acids, and Long-Chain Bases.- 1.3.3. Determination of Carbohydrate Composition.- 1.3.4. Release of Oligosaccharides from Glycosphingolipids.- 1.3.5. Determination of Carbohydrate Sequence.- 1.3.6. Determination of the Position of Glycosyl Linkages.- 1.3.7. Determination of Anomeric Configuration (a or ß) in Glycolipids by Chromium Trioxide Oxidation.- 1.3.8. Direct-Probe Mass Spectrometry of Glycolipids.- 1.3.9. Spectrometric Analysis of Glycosphingolipids: Infrared (IR), Nuclear Magnetic Resonance, and Electron Spin Resonance Spectra.- 1.4. Structure of Glycosphingolipids.- 1.4.1. Structural Variation in Ceramides.- 1.4.2. Simpler Glycosphingolipids: Ceramide Monohexosides, Ceramide Dihexosides, and Sulfatides.- 1.4.3. Globo-Series Glycolipids.- 1.4.4. Lacto-Series Glycolipids.- 1.4.5. Muco-Series Glycolipids: Glycolipids with Digalactosyl to Hexagalactosyl Core Structure.- 1.4.6. Simpler Gangliosides and Hematosides: Sialosyl Glycolipids without Hexosamines.- 1.4.7. Ganglio-Series Glycolipids: Gangliosides with Ganglio-N- triose, Ganglio-N-tetraose, and Ganglio-N-pentaose Structure.- 1.4.8. Glycosphingolipids of Water-Living Invertebrates.- 1.4.9. Plant Sphingolipids (Phytoglycosphingolipids).- 1.5. Chemical Synthesis and Modification of Sphingosines and Glycosphingolipids.- 1.5.1. Synthesis of Long-Chain Bases.- 1.5.2. Synthesis of Glycosphingolipids.- 1.6. Pioneers in Glycolipid Chemistry.- 1.7. References.- 2 Sphingolipid Metabolism.- 2.1. Sphingosine Bases.- 2.1.1. In Vivo Studies.- 2.1.2. In Vivo Studies.- 2.1.3. In Vivo Studies on Sphingosine Base Utilization.- 2.2. The Psychosines.- 2.2.1. Galactosylsphingosine Formation.- 2.2.2. Glucosylsphingosine Formation.- 2.2.3. Galactosylsphingosine Acylation.- 2.2.4. Glucosylsphingosine Acylation.- 2.3. Ceramide (N-Acylsphingosine).- 2.4. Galactosylceramide.- 2.4.1. In Vivo Studies.- 2.4.2. Biosynthesis in Vivo.- 2.4.3. Hydrolysis in Vivo.- 2.5. Ceramide-galactoside-3-sulfate (Sulfatide).- 2.5.1. In Vivo Studies.- 2.5.2. Biosynthesis in Vivo.- 2.5.3. Hydrolysis in Vivo.- 2.6. Glucosylceramide.- 2.6.1. Biosynthesis in Vivo.- 2.6.2. Hydrolysis in Vivo.- 2.7. Ganglioside Metabolism.- 2.7.1. In Vivo Studies.- 2.7.2. Biosynthesis m VeVrö.- 2.8. Sphingomyelin.- 2.8.1. Biosynthesis in Vivo.- 2.8.2. Hydrolysis in Vivo.- 2.9. Biosynthesis of the Sphingolipids: Summary.- 2.10. Hydrolysis of the SpWgolipids: Summary.- 2.11. References.- 3 The Sphingolipidoses.- 3.1. Farber s Lipogranulomatosis.- 3.1.1. Chemical Studies.- 3.1.2. Enzyme Defect.- 3.2. Krabbe s Disease (Globoid Cell Leukodystrophy).- 3.2.1. Clinical.- 3.2.2. Pathology.- 3.2.3. Biochemistry.- 3.3. Metachromatic Leukodystrophy.- 3.3.1. Clinical.- 3.3.2. Pathology.- 3.3.3. Biochemistry.- 3.4. Gaucher s Disease.- 3.4.1. Clinical.- 3.4.2. Pathology.- 3.4.3. Biochemistry.- 3.5. Fabry s Disease (Angiokeratoma Corporis Diffusium).- 3.5.1. Clinical.- 3.5.2. Pathology.- 3.5.3. Biochemistry.- 3.6. The Gangliosidoses.- 3.6.1. GM2 Gangliosidosis Type I (Classical Tay-Sachs Disease).- 3.6.2. GM2 Gangliosidosis Type II (Sandhoff-Jatzkewitz Variant).- 3.6.3. GM2 Gangliosidosis Type III (Juvenile).- 3.6.4. GM1 Gangliosidosis Type I (Pseudo-Hurler s, Landing s Syndrome; Neurovisual Lipidosis).- 3.6.5. GM1 Gangliosidosis Type II (Juvenile GMi Gangliosidosis; Derry s Syndrome).- 3.7. Niemann-Pick s Disease.- 3.7.1. Clinical.- 3.7.2. Pathology.- 3.7.3. Biochemistry.- 3.8. GM
1 Chemistry of Glycosphingolipids.- 1.1. Introduction.- 1.1.1. Classification and Nomenclature of Glycosphingolipids.- 1.1.2. Brief History of the Chemistry of Glycosphingolipids.- 1.2. Isolation of Glycosphingolipids.- 1.2.1. Extraction.- 1.2.2. Purification of Lipid Extract: Elimination of Nonlipid Contaminants.- 1.2.3. Separation of Gangliosides and Long-Chain Neutral Glycolipids.- 1.2.4. Separation of Individual Glycolipids.- 1.3. Characterization of Glycosphingolipids.- 1.3.1. Analysis by Thin-Layer Chromatography.- 1.3.2. Characterization of Ceramide Structure, Fatty Acids, and Long-Chain Bases.- 1.3.3. Determination of Carbohydrate Composition.- 1.3.4. Release of Oligosaccharides from Glycosphingolipids.- 1.3.5. Determination of Carbohydrate Sequence.- 1.3.6. Determination of the Position of Glycosyl Linkages.- 1.3.7. Determination of Anomeric Configuration (a or ß) in Glycolipids by Chromium Trioxide Oxidation.- 1.3.8. Direct-Probe Mass Spectrometry of Glycolipids.- 1.3.9. Spectrometric Analysis of Glycosphingolipids: Infrared (IR), Nuclear Magnetic Resonance, and Electron Spin Resonance Spectra.- 1.4. Structure of Glycosphingolipids.- 1.4.1. Structural Variation in Ceramides.- 1.4.2. Simpler Glycosphingolipids: Ceramide Monohexosides, Ceramide Dihexosides, and Sulfatides.- 1.4.3. Globo-Series Glycolipids.- 1.4.4. Lacto-Series Glycolipids.- 1.4.5. Muco-Series Glycolipids: Glycolipids with Digalactosyl to Hexagalactosyl Core Structure.- 1.4.6. Simpler Gangliosides and Hematosides: Sialosyl Glycolipids without Hexosamines.- 1.4.7. Ganglio-Series Glycolipids: Gangliosides with Ganglio-N- triose, Ganglio-N-tetraose, and Ganglio-N-pentaose Structure.- 1.4.8. Glycosphingolipids of Water-Living Invertebrates.- 1.4.9. Plant Sphingolipids (Phytoglycosphingolipids).- 1.5. Chemical Synthesis and Modification of Sphingosines and Glycosphingolipids.- 1.5.1. Synthesis of Long-Chain Bases.- 1.5.2. Synthesis of Glycosphingolipids.- 1.6. Pioneers in Glycolipid Chemistry.- 1.7. References.- 2 Sphingolipid Metabolism.- 2.1. Sphingosine Bases.- 2.1.1. In Vivo Studies.- 2.1.2. In Vivo Studies.- 2.1.3. In Vivo Studies on Sphingosine Base Utilization.- 2.2. The Psychosines.- 2.2.1. Galactosylsphingosine Formation.- 2.2.2. Glucosylsphingosine Formation.- 2.2.3. Galactosylsphingosine Acylation.- 2.2.4. Glucosylsphingosine Acylation.- 2.3. Ceramide (N-Acylsphingosine).- 2.4. Galactosylceramide.- 2.4.1. In Vivo Studies.- 2.4.2. Biosynthesis in Vivo.- 2.4.3. Hydrolysis in Vivo.- 2.5. Ceramide-galactoside-3-sulfate (Sulfatide).- 2.5.1. In Vivo Studies.- 2.5.2. Biosynthesis in Vivo.- 2.5.3. Hydrolysis in Vivo.- 2.6. Glucosylceramide.- 2.6.1. Biosynthesis in Vivo.- 2.6.2. Hydrolysis in Vivo.- 2.7. Ganglioside Metabolism.- 2.7.1. In Vivo Studies.- 2.7.2. Biosynthesis m VeVrö.- 2.8. Sphingomyelin.- 2.8.1. Biosynthesis in Vivo.- 2.8.2. Hydrolysis in Vivo.- 2.9. Biosynthesis of the Sphingolipids: Summary.- 2.10. Hydrolysis of the SpWgolipids: Summary.- 2.11. References.- 3 The Sphingolipidoses.- 3.1. Farber s Lipogranulomatosis.- 3.1.1. Chemical Studies.- 3.1.2. Enzyme Defect.- 3.2. Krabbe s Disease (Globoid Cell Leukodystrophy).- 3.2.1. Clinical.- 3.2.2. Pathology.- 3.2.3. Biochemistry.- 3.3. Metachromatic Leukodystrophy.- 3.3.1. Clinical.- 3.3.2. Pathology.- 3.3.3. Biochemistry.- 3.4. Gaucher s Disease.- 3.4.1. Clinical.- 3.4.2. Pathology.- 3.4.3. Biochemistry.- 3.5. Fabry s Disease (Angiokeratoma Corporis Diffusium).- 3.5.1. Clinical.- 3.5.2. Pathology.- 3.5.3. Biochemistry.- 3.6. The Gangliosidoses.- 3.6.1. GM2 Gangliosidosis Type I (Classical Tay-Sachs Disease).- 3.6.2. GM2 Gangliosidosis Type II (Sandhoff-Jatzkewitz Variant).- 3.6.3. GM2 Gangliosidosis Type III (Juvenile).- 3.6.4. GM1 Gangliosidosis Type I (Pseudo-Hurler s, Landing s Syndrome; Neurovisual Lipidosis).- 3.6.5. GM1 Gangliosidosis Type II (Juvenile GMi Gangliosidosis; Derry s Syndrome).- 3.7. Niemann-Pick s Disease.- 3.7.1. Clinical.- 3.7.2. Pathology.- 3.7.3. Biochemistry.- 3.8. GM
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