I. Lipids.- Biochemistry, Physiology, Methodology.- The Chemistry of Mammalian Lipids.- A. Simple Lipids.- I. Glycerides.- 1. Monoglycerides.- 2. D- ?, ß-Diglycerides.- 3. Triglycerides.- II. Cholesterol and Cholesterol Esters.- III. Büe Acids.- B. Complex Lipids.- I. Glycerophospholipids.- 1. Phosphatidic Acids.- 2. Phosphatidyl Cholines (Lecithins).- 3. Phosphatidyl Ethanolamines (Cephalins).- 4. Phosphatidyl Serines.- 5. Lyso-glycerophospholipids.- 6. Cardiolipin.- 7. Phosphatidyl Glycerol.- 8. Phosphatidyl Inositols.- 9. Choline and Ethanolamine Plasmalogens.- 10. Glyceryl Ether Phospholipids.- II. Sphingolipids.- 1. Sphingomyelin.- 2. Glycosphingolipids.- a) Cerebrosides.- b) Sulfatides.- c) Ceramide-polyhexosides.- d) Gangliosides.- III. Fatty Acids and Fatty Aldehydes.- IV. Prostaglandins.- References.- C. Fatty Acid Oxydation.- I. ß-Oxydation of Saturated Fatty Acids.- II ß- Oxydation of Unsaturated Fatty Acids.- III. a-Oxydation.- D. Biosynthesis of Fatty Acids.- I. Biosynthesis of Saturated Fatty Acids.- II. Biosynthesis of Mono- and Polyunsaturated Fatty Acids.- a) Monoenoic Acids.- b) Polyunsaturated Fatty Acids.- c) Control Mechanisms of Fatty Acid Synthesis.- References.- Biochemistry of Triglycerides.- I. Absorption from the Intestine.- 1. Lipolysis in the Lumen.- a) Pancreatic Lipase.- b) The Extent of Hydrolysis.- c) Emulsification.- 2. Uptake and Metabolism in the Intestinal Cells.- a) Uptake.- b) Resynthesis of Triglycerides in Mucosal Cells.- c) Phospholipids.- d) Fate of Short Chain Fatty Acids.- II. Metabolism of Lymph and Blood Chylomicra.- 1. Rate and Site of Removal.- 2. Clearing Factor and the Mechanism of Uptake by the Tissues.- III. Metabolism of Triglycerides in the Liver.- 1. Uptake and Release.- 2. Synthesis of Triglycerides.- 3. Fatty Livers.- IV. Metabolism of Triglycerides in Adipose Tissue.- 1. Deposition of Triglycerides.- 2. Mobilization of Triglycerides.- References.- Biochemistry of Steroids.- I. Biosynthesis.- 1. The Biosynthesis of Cholesterol.- 2. Other Sterols.- II. Conversion of Cholesterol to Other Steroids.- 1. Bile Acids.- 2. Adrenocortical Steroid Hormones.- 3. Sex Hormones.- 4. Neutral Sterols.- References.- Biochemistry of Phosphatides.- I. Biosynthesis of Glycerophosphatides.- 1. Phosphatide Acid.- 2. Phosphatidyl Choline (Lecithin).- 3. Choline Plasmalogen.- 4. Phosphatidyl Ethanolamine.- 5. Ethanolamine Plasmalogen.- 6. Glycerol Ether Phosphatides.- 7. Phosphatidyl Serine.- 8. Phosphatidyl Inositol.- 9. Polyphosphoinositides.- 10. hosphatidyl Glycerol.- 11. Cardiolipin.- II. Degradation of Glycerophosphates.- 1. Phosphatidyl Choline (Lecithin).- a) Phospholipase A.- b) Phospholipase B (Lysophospholipase).- c) Phospholipase C.- d) Phospholipase D.- e) GPC Diesterase.- f) Phosphomonoesterases.- 2. Phosphatidyl Ethanolamine.- 3. Phosphatidyl Serine.- 4. Phosphatidyl Glycerol.- 5. Cardiolipin.- 6.Phosphatide Acid.- 7. Plasmalogens.- 8. Phosphatidyl Inositol.- 9.Polyphosphoinositides.- III. Conclusion.- References.- Biochemistry of Sphingosine Containing Lipids.- Nomenclature.- I. Studies on the Synthesis of Sphingolipids.- Sphingosine and Dihydrosphingosine.- II. Studies on the Intact Animal.- 1. Developmental Studies.- 2. Studies Conducted in Vivo with Isotopes.- a) Sphingosine.- b) Carbohydrates.- c) Sulfate.- d) Fatty Acids.- 3. Cerebroside and Ceramide Metabolism.- a) Turnover Studies.- b) Metabolic Pathways.- c) Immunology of the Sphingolipids.- d) Diseases Involving Sphingolipids.- References.- Lipoproteins.- Composition and Properties of Plasma Lipoproteins.- 1. Definition of a Lipoprotein Class.- 2. Homogeneity: Distribution in Size and Shape.- 3. Chylomicrons.- 4. Very Low Density and Low Density Lipoproteins.- 5. High Density Lipoproteins.- 6. The Analysis of Chylomicrons and Lipoproteins.- References.- Methods for Separation and Determination of Lipids.- I. Isolation of Lipids.- II. Quantitative Determination of Total Lipids.- III. Separation of Lipid Classes.- a) Fractionation into Polar and Unpolar Lipids.- b) Fractionation into Main Lipid Classes.- IV. Separation of Individual Members of Lipid Classes.- V. Quantitative Detemiination of Lipid Classes.- a) Cholesterol and Cholesterol Esters.- b) Phospholipids.- c) Glycerides.- d) Total Fatty Acids.- e) Free Fatty Acids.- f) Bile Acids.- g) Other Lipids.- VI. Determination of Lipoproteins.- VII. Determination of Lipoprotein Lipase Activity.- VIII. Normal Lipid Concentrations.- References.- II. Lipidoses.- Clinic, Pathology, Pathophysiology, Genetics.- Gangliosidoses.- I. Congenital Amaurotic Family Idiocy.- Definition and Introduction.- Clinical Manifestations.- Pathology.- Results of Lipid Analyses.- Discussion.- Diagnosis.- II. Infantile Amaurotic Family Idiocy (Tay-Sachs Disease).- Definition and Introduction.- Historical Review.- Incidence (Age, Sex, Race).- Clinical Manifestations.- Electroencephalography.- Cerebrospinal Fluid.- Serum.- Enzymes in Plasma and Cerebrospinal Fluid.- Pathology.- Brain.- Eye.- Other Organs.- Histology.- Membranous Cytoplasmic Bodies.- Histochemistry.- Results of Lipid Analyses.- Brain.- The Gangliosides in Tay-Sachs Disease.- Diagnosis.- Pathogenetic Aspects.- III. Late-Infantile Amaurotic Family Idiocy.- Definition and Introduction.- Clinical Manifestations.- Pathology.- Results of Lipid Analyses.- IV. Juvenile Amaurotic Family Idiocy.- Definition and Introduction.- Incidence (Age, Sex, Race).- Clinical Manifestations.- Cerebrospinal Fluid.- Electroencephalography.- Hematology.- Serum.- Pathology.- The Stored Material.- V. Adult Amaurotic Family Idiocy.- Definition and Introduction.- Results of Lipid Analyses.- VI. Neurovisceral Gangliosidoses.- Definition and Introduction.- Case Reports.- Results of Lipid Analyses.- VII. Hurler's Disease (Gargoylism).- Definition and Introduction.- Clinical Manifestations.- Laboratory Findings.- Pathology.- Results of Lipid Analyses.- Therapy of Gangliosidoses.- References.- Gaucher's Disease.- Definition and Introduction.- Historical Review.- Incidence (Age, Sex, Race).- Clinical Manifestations.- Hematology.- Other Laboratory Findings.- Pathology.- Gaucher Cells.- Spleen.- Liver.- Lymph Nodes.- Bones and Bone Marrow.- Other Organs.- Central Nervous System.- Results of Lipid Analyses.- Serum Lipids.- Brain Lipids.- Tissue Lipids.- Chemistry of the Stored Cerebroside.- Sphingosine.- Fatty Acid Pattern.- Hexose.- Pathogenetic Considerations.- Treatment.- References.- Niemann-Pick Disease.- Definition and Introduction.- Historical Review.- Incidence (Age, Sex, Race).- Clinical Manifestations.- Neurology.- Hematology.- Other Laboratory Findings.- Bones.- Skin.- Eyes.- Course and Prognosis.- Pathology.- Niemann-Pick Cells.- Spleen.- Liver.- Lymph Nodes.- Bone Marrow.- Thymus.- Lungs.- Kidneys.- Other Organs.- Central Nervous System.- Results of Lipid Analyses.- Brain Lipids.- Other Organs.- Serum Lipids.- Pathogenetic Aspects.- Diagnosis.- Treatment.- References.- Metachromatic Leucodystrophy.- Definition and Introduction.- Historical Review.- Incidence (Age, Sex, Race).- Clinical Manifestations.- Cerebrospinal Fluid.- Blood.- Urine.- Cholecystography.- Pathology.- Central Nervous System.- Extraneural Tissues.- Kidneys.- Gallbladder.- Other Organs.- Histochemical Findings.- Results of Lipid Analyses.- Brain.- Other Organs.- The Stored Lipid.- The Normal Sulfatides.- Sulfatides in Metachromatic Leucodystrophy.- Diagnosis.- Pathogenetic Aspects.- References.- Angiokeratoma Corporis Diffusum (Fabry's Disease).- Definition and Introduction.- Historical Review.- Incidence (Age, Sex, Race).- Clinical Manifestations.- Skin Lesions.- Kidneys.- Cardiac Disturbances.- Edema.- Neurologic Disturbances.- Eyes.- Other Signs and Symptoms.- Serum.- Hematology.- Urine.- Pathology.- Vasculature.- Kidneys.- Heart and Skeletal Muscle.- Skin.- Nervous System.- Reticuloendothelial Tissues.- Other Organs.- Results of Lipid Analyses.- Pathogenetic Aspects.- Diagnosis.- Treatmen…