Hepatic Lipase Deficiency

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.