Postprandial Fatty Acid Metabolism in Subjects With Lipoprotein Lipase Deficiency

Inclusion Criteria: * 8 healthy LPL-deficient individuals (LPLD subjects) with history of fasting TG \> 5 mmol/l and homozygote or compound heterozygote for a LPL-gene mutation; * 8 control subjects (fasting glucose \< 5.6, 2-hour post 75g OGTT glucose \< 7.8 mmol/l and HbA1c \< 5.8%; fasting TG \< 1.5 mmol/l); * age 18 to 75 yo; * To be willing and able to adhere to the specifications of the protocol; * To have signed an informed consent document indicating that they understood the purpose Exclusion Criteria: * age \< 18 yo; * overt cardiovascular disease as assessed by medical history, physical exam, and abnormal ECG * Treatment with a fibrate, thiazolidinedione, beta-blocker or other drug known to affect lipid or carbohydrate metabolism (except statins, metformin, and other antihypertensive agents that can be safely interrupted); * Treatment with anti-hypertensive medication (only for LPL-deficient individuals); * presence of liver or renal disease; uncontrolled thyroid disorder; * previous diagnosis of heparin-induced thrombocytopenia; * Treatment with oral anticoagulation medication or platelet aggregation inhibiting drugs; * A history of major hemorrhagic event; * smoking (\>1 cigarette/day) and/or consumption of \>2 alcoholic beverages per day;; * Female of child-bearing potential who is pregnant, breast feeding or intends to become pregnant or pre-menopausal female with a positive serum pregnancy test at the time of enrollment.