Subject(s)
Hypolipoproteinemias , Anticholesteremic Agents/adverse effects , Apolipoprotein A-I/deficiency , Apolipoprotein A-I/genetics , Blood Protein Disorders/complications , Blood Protein Disorders/metabolism , Hematologic Diseases/complications , Hematologic Diseases/metabolism , Humans , Hypobetalipoproteinemias/classification , Hypobetalipoproteinemias/etiology , Hypolipoproteinemias/classification , Hypolipoproteinemias/etiology , Lipid Metabolism , Liver Diseases/complications , Liver Diseases/metabolism , Lymphokines/adverse effects , Mutation , Neoplasms/complications , Neoplasms/metabolism , Probucol/adverse effects , Tangier Disease , Thyroid Diseases/complications , Thyroid Diseases/metabolismABSTRACT
There is increasing evidence that high-density lipoprotein (HDL) and its subfractions are protective against atherosclerotic cardiovascular disease. Physical exercise, weight reduction, smoking cessation, diabetes mellitus control, and specific drugs, including niacin, fibrates, and estrogens, are effective methods to increase HDL levels. Niacin is the oldest and most powerful clinical agent for raising HDL levels. Niaspan, an extended-release niacin formulation, is as potent as immediate-release niacin in increasing levels of HDL cholesterol; subfractions HDL2 and HDL3; apolipoprotein A-I, the major protein of HDL, and its cardioprotective subfraction lipoprotein A-I. Recent research from our laboratory suggests a novel mechanism by which niacin inhibits hepatic removal of HDL-apoprotein A-I without interfering with the removal of cholesterol carried by HDL, thus augmenting reverse cholesterol transport. Other mechanistic studies indicate that fibrates and estrogens stimulate the synthesis and production of HDL-apoprotein A-I. Because niacin decreases HDL-apoprotein A-I removal, and fibrates and estrogens increase HDL-apoprotein A-I production, combinations of niacin with these agents may raise HDL levels more than fibrates or estrogens alone.
Subject(s)
Hypolipoproteinemias/drug therapy , Lipoproteins, HDL/drug effects , Niacin/pharmacology , Apolipoprotein A-I/drug effects , Apolipoprotein A-I/metabolism , Delayed-Action Preparations , Humans , Hypolipoproteinemias/classification , Lipoproteins, HDL/blood , Lipoproteins, HDL/metabolismABSTRACT
The article defines apolipoprotein diseases as the structural defects of apolipoproteins or disorders in their synthesis or secretion. The survey covers a range of atherogenous defects, providing a detailed description of their clinical-biochemical and genetic aspects as well as diagnostic and differential diagnostic criteria. Attention is paid also to the significance of analytical isoelectric focusing.
Subject(s)
Apolipoproteins/chemistry , Arteriosclerosis/etiology , Abetalipoproteinemia/classification , Abetalipoproteinemia/complications , Apolipoproteins/blood , Apolipoproteins/genetics , Female , Humans , Hypolipoproteinemias/classification , Hypolipoproteinemias/complications , MaleSubject(s)
Hyperlipoproteinemias/etiology , Hypolipoproteinemias/etiology , Biological Transport , Black People , Coronary Disease/etiology , Humans , Hyperlipoproteinemias/classification , Hyperlipoproteinemias/complications , Hypolipoproteinemias/classification , Hypolipoproteinemias/complications , Lipid Metabolism , Risk , Vascular Diseases/etiology , White PeopleSubject(s)
Hyperlipidemias/diagnosis , Hyperlipoproteinemias/diagnosis , Hypolipoproteinemias/diagnosis , Adolescent , Child , Child, Preschool , Humans , Hyperlipidemias/blood , Hyperlipidemias/classification , Hyperlipoproteinemias/blood , Hyperlipoproteinemias/classification , Hypolipoproteinemias/blood , Hypolipoproteinemias/classification , Infant , Infant, Newborn , Lipids/blood , Lipoproteins/blood , Obesity/blood , Obesity/diagnosisSubject(s)
Hyperlipoproteinemias/classification , Hypolipoproteinemias/classification , Apolipoproteins/deficiency , Apolipoproteins/genetics , Genetic Variation , Humans , Hyperlipoproteinemias/genetics , Hyperlipoproteinemias/metabolism , Hypolipoproteinemias/genetics , Hypolipoproteinemias/metabolism , Lecithin Cholesterol Acyltransferase Deficiency/classification , Mutation , Receptors, LDL/metabolismSubject(s)
Coronary Disease/etiology , Lipoproteins/blood , Receptors, Lipoprotein , Amino Acid Sequence , Apolipoproteins/blood , Apoproteins/blood , Arteriosclerosis/etiology , Chemical Phenomena , Chemistry , Humans , Hyperlipoproteinemias/classification , Hypolipoproteinemias/classification , Lipase/metabolism , Lipoprotein Lipase/metabolism , Lipoproteins, HDL/blood , Lipoproteins, LDL/blood , Lipoproteins, VLDL/blood , Low Density Lipoprotein Receptor-Related Protein-1 , Phosphatidylcholine-Sterol O-Acyltransferase/metabolism , Receptors, Cell Surface/metabolism , Risk , Terminology as TopicABSTRACT
A patient is reported with hypobetalipoproteinaemia and clinical features resembling the Bassen-Kornzweig syndrome (abetalipoproteinaemia) more completely than previously described. This supports a link between hypobetalipoproteinaemia and abetalipoproteinaemia and it is suggested that the Bassen-Kornzweig syndrome has a wide spectrum with serum betalipoprotein ranging from absent to normal. It is likely that there are different genetic entities with similar end results.
