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Receptor-Mediated Low Density Lipoprotein Metabolism

  • Chapter
Atherosclerosis

Part of the book series: Altschul Symposia Series ((ALSS,volume 1))

Abstract

The concept of receptor-mediated metabolism of lipoproteins emerged 17 years ago from studies on human skin fibroblasts grown in culture1. These experiments were designed to elucidate the normal function of low density lipoprotein (LDL), about which little was known at that time. Biochemical studies showed that a specific cell surface receptor, the LDL receptor, mediates the binding, uptake and degradation of LDL, thus supplying almost all cells in the body with cholesterol. Detailed insight into the molecular mechanisms underlying this complex process was obtained from studies with fibroblasts derived from patients with the phenotype of homozygous familial hypercholesterolemia (FH). As in many other biological systems, the expression of a disease state in a defined cellular system was essential to the discovery of the causal factor: FH is now one of the best characterized genetic diseases at the molecular level. As will be outlined below, several groups of mutations occur naturally in the structural gene for the LDL receptor which disrupt its normal function and lead to severe hypercholesterolemia, myocardial infarctions and premature atherosclerosis. Thus, the important role of lipoprotein receptors in normal physiology is underscored by the dramatic consequences of their functional absence.

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Author information

Authors and Affiliations

  1. Lipid and Lipoprotein Research Group and Department of Biochemistry 327 Heritage Medical Research Centre, University of Alberta, Edmonton, Alberta, T6G 2S2, Canada

    Wolfgang J. Schneider

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  1. Wolfgang J. Schneider
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Editors and Affiliations

  1. University of Toronto, Toronto, Ontario, Canada

    Avrum I. Gotlieb  & B. Lowell Langille  & 

  2. University of Saskatchewan, Saskatoon, Saskatchewan, Canada

    Sergey Fedoroff

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© 1991 Springer Science+Business Media New York

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Schneider, W.J. (1991). Receptor-Mediated Low Density Lipoprotein Metabolism. In: Gotlieb, A.I., Langille, B.L., Fedoroff, S. (eds) Atherosclerosis. Altschul Symposia Series, vol 1. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-3754-0_18

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  • DOI: https://doi.org/10.1007/978-1-4615-3754-0_18

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4613-6672-0

  • Online ISBN: 978-1-4615-3754-0

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