Current interest in lipoprotein deficiency states stems from the growing realization of their importance in the etiology of premature coronary heart disease. While hypercholesterolemia and coronary heart disease risk are strongly correlated in their etiologic relationship, it is becoming equally clear that deficiencies in HDL, whether congenital or acquired, also enhance the risk for the future development of coronary atherosclerosis. This has led to renewed attention to the lipid hypothesis and realization of the fact that each lipoprotein class and apoprotein species has specific functions in the transport and cellular uptake of various lipids. It is a truism that a biochemical correlate of disease once identified is subsequently recognized with increasing frequency in clinical medicine. The story of HDL was no exception. Indeed hypoalphalipoproteinemia appears to be a disease of high prevalence approaching and perhaps even exceeding that of familial hypercholesterolemia. Its clinical signifi­ cance escaped our notice for many years largely due to a heavy emphasis on hypercholesterolemia and to difficulties in measuring HDL reliably.

One: Lipoprotein Deficiency and Atherosclerosis.- Genetic High Density Lipoprotein Deficiency States and Atherosclerosis.- Metabolic Interrelationships of HDL Subclasses.- Speciation of HDL.- Modifications and Degradation of High Density Lipoproteins.- Effect of Diabetes Mellitus and End-Stage Renal Disease on HDL Metabolism.- HDL Binding to Human Adipocyte Plasma Membranes: Regional Variation in Omental and Subcutaneous Depots.- Clinical, Nutritional and Biochemical Consequences of Apolipoprotein B Deficiency.- Two: Hypoalphalipoproteinemia Syndromes.- Familial Hypoalphalipoproteinemia.- A Family Study of Hypoalphalipoproteinemia.- Apoprotein A-1 and Lecithin: Cholesterol Acyltransferase in a Patient With Tangier Disease.- Severe Hypoalphalipoproteinemia Induced by a Combination of Probucol and Clofibrate.- Control of Plasma HDL Levels After Plasmapheresis.- Clinical Syndrome and Lipid Metabolism in Hereditary Deficiency of Apolipoproteins A-1 and C-III, Variant I.- The Molecular Biology of Human Apo A-1, Apo A-II, Apo C-II and Apo B.- Three: LCAT and Cholesterol Transport.- Lecithin: Cholesterol Acyltransferase, A Review and Immunochemical Studies.- Lecithin: Cholesterol Acyltransferase Deficiency Syndromes.- Cholesterol Efflux: Mechanism and Regulation.- Inhibition and Activation of Cholesteryl Ester Transfer and its Significance in Plasma Cholesterol Metabolism.- Four: Hypertriglyceridemia Secondary to Enzyme or Apoprotein Deficiency.- Apolipoprotein C-II Deficiency.- Primary Lipoprotein Lipase Deficiency.- The Genetics and Molecular Biology of Apolipoprotein C-II.- Familial Hepatic Lipase Deficiency.- The Apo E-System: Genetic Control of Plasma Lipoprotein Concentration.- Type III Hyperlipoproteinemia: A Focus on Lipoprotein Receptor-Apolipoprotein E2 Interactions.- The Role of Apolipoprotein E in Modulating the Metabolism of Apolipoprotein B-48 and Apolipoprotein B-100 Containing Lipoproteins in Humans.