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Primary Lipoprotein Lipase Deficiency

  • Chapter
Lipoprotein Deficiency Syndromes

Part of the book series: Advances in Experimetal Medicine and Biology ((AEMB,volume 201))

Abstract

The enzyme lipoprotein lipase (LPL) is a glycoprotein located on the luminal surface of capillary endothelial cells (see reviews: 1–4). It is bound to a glycosaminoglycan on the endothelium, can be displaced into plasma by intravenous heparin or other polyanions, and binds to heparin-Sepharose gels. The enzyme has an apparent monomeric molecular weight on SDS gel of over 60,000 and 48,300 by sedimentation-equilibrium ultracentrifugation, and probably functions as a dimer in vivo. It has binding sites for heparin, for the cofactor apolipoprotein CII, and for lipid, and has a separate catalytic site for triglyceride hydrolysis. It is inhibited by serine proteases inhibitors, by protamine, and by high ionic strength. The enzyme appears to be synthesized in a number of different parenchymal cells including monocyte-derived macrophages, Kupfer cells, adipocytes, and cells in cardiac and skeletal muscle. The enzyme is secreted from the adipocyte and transported in an unknown fashion to the plasma surface of the capillary endothelial cell, where it has several functions in humans.

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Author information

Authors and Affiliations

  1. University of Washington, Seattle, USA

    John D. Brunzell, Per-Henrik Iverius, Mark S. Scheibel & Wilfred Y. Fujimoto

  2. University of British Columbia, Vancouver, Canada

    Michael R. Hayden, Roger McLeod & Jiri Frolich

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  1. John D. Brunzell
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Editors and Affiliations

  1. Institute of Medical Sciences, University of Toronto, Toronto, Ontario, Canada

    Aubie Angel

  2. Shaughnessy Hospital, University of British Columbia, Vancouver, British Columbia, Canada

    Jiri Frohlich

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Brunzell, J.D. et al. (1986). Primary Lipoprotein Lipase Deficiency. In: Angel, A., Frohlich, J. (eds) Lipoprotein Deficiency Syndromes. Advances in Experimetal Medicine and Biology, vol 201. Springer, Boston, MA. https://doi.org/10.1007/978-1-4684-1262-8_20

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  • DOI: https://doi.org/10.1007/978-1-4684-1262-8_20

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4684-1264-2

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