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2288 pages added, reviewed or updated during the last month (last updated: 19/4/2021)


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Hepatic Lipase Deficiency

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HDL Metabolism in Hepatic Lipase Deficiency

  • in normal high-density lipoprotein (HDL) metabolism, the action of hepatic lipase on triglyceride-rich HDL results in the production of small phospholipid-rich HDL species (ie, HDL3)
  • hepatic lipase deficiency therefore delays the catabolism of triglyceride-rich HDL (ie, HDL2), resulting in modest elevations in HDL-cholesterol and apolipoprotein A-I and in variable elevations in total cholesterol and triglycerides
  • genetic deficiency of hepatic lipase also impairs the metabolism of very-low-density lipoproteins (VLDLs) and intermediate-density lipoproteins (IDLs), leading to their accumulation in plasma

Reference:

  1. Breslow JL. Familial disorders of high-density lipoprotein metabolism. In: Scriver CR, Beaudet AL, Sly WS, Valle D, eds. The Metabolic and Molecular Bases of Inherited Disease. 7th ed. New York: McGraw-Hill; 1995:2031–2052.
  2. Connelly PW, Hegele RA. Hepatic lipase deficiency. Crit Rev Clin Lab Sci. 1998;35:547–572.
  3. Rader DJ. Lipid disorders. In: Topol EJ, ed. Textbook of Cardiovascular Medicine. Philadelphia: Lippincott-Raven; 1998:59–90.

Last reviewed 01/2018

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