Lipoprotein lipase hydrolyzes triglycerides in chylomicrons, very low density lipoproteins (VLDLs), and intermediate density lipoproteins (IDLs) to release free fatty acids for cellular absorption. A deficiency in the enzyme can cause a dramatic elevation in chylomicron and triglyceride levels. VLDL concentration is normal, but the low density lipoproteins (LDLs) and high density lipoproteins (HDLs) are abnormally low.

The risk of atherosclerosis is not elevated.