Postprandial Fatty Acid Metabolism in Subjects With Lipoprotein Lipase Deficiency

Inclusion Criteria:

• 8 healthy LPL-deficient individuals (LPLD subjects) with history of fasting TG > 5mmol/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/land 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 thepurpose

Exclusion Criteria:

• age < 18 yo;

• overt cardiovascular disease as assessed by medical history, physical exam, andabnormal ECG

• Treatment with a fibrate, thiazolidinedione, beta-blocker or other drug known toaffect lipid or carbohydrate metabolism (except statins, metformin, and otherantihypertensive 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 inhibitingdrugs;

• 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 tobecome pregnant or pre-menopausal female with a positive serum pregnancy test at thetime of enrollment.