Genetic High Density Lipoprotein Deficiency States and Atherosclerosis

  • Ernst J. Schaefer
  • Judith R. McNamara
  • Carol J. Mitri
  • Jose M. Ordovas
Part of the Advances in Experimetal Medicine and Biology book series (AEMB, volume 201)


High density, lipoprotein (HDL) as found in human plasma have a density of 1.063–1.21 g/ml, and are composed (weight percent) of approximately 50% protein, 25% phospholipid, 20% cholesterol, and 5% triglyceride (1). Fluctuations in HDL levels have been associated mainly with alterations in HDL2 (density, 1.063–1.125 g/ml), rather than HDL3 (density, 1.125–1.21 g/ml (2). Apolipoproteins (apo) A-I and A-II are the major proteins of HDL. Minor constituents include apoB, apoC-I, apoC-II, apoC-III, apoD, apoE, apoF, apoG, apoLp(a) (3–12). Apolipoprotein not only have structural roles in HDL particles, but have other functions as well. ApoA-I and apoC-I have both been reported to activate lecithin/cholesterol acyltransferase (LCAT), while apoA-II has been reported to enhance hepatic lipase activity (12–15). ApoC-II activates the enzyme lipoprotein lipase, while apoC-III has been shown to inhibit hepatic chylomicron remnant uptake (16,17). Both apoB and apoE can bind to the apoB,E receptor (LDL receptor) on various cell surfaces, while apoE also binds to the liver apoE receptor, which is essential for chylomicron remnant uptake (18,19). The low density lipoprotein (LDL) receptor appears to be crucial for regulating LDL levels in plasma, as well as for maintaining intracellular cholesterol homeostasis. HDL particles can bind to specific sites on various cell surfaces (20).


Cholesterol Ester Premature Coronary Artery Disease Tangier Disease Intracellular Cholesterol Homeostasis Familial LCAT Deficiency 
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Copyright information

© Springer Science+Business Media New York 1986

Authors and Affiliations

  • Ernst J. Schaefer
    • 1
  • Judith R. McNamara
    • 1
  • Carol J. Mitri
    • 1
  • Jose M. Ordovas
    • 1
  1. 1.Lipid Metabolism LaboratoryHuman Nutrition Research Center on Aging at Tufts UniversityBostonUSA

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