Which enzyme deficiency is primarily associated with familial hypertriglyceridemia?

Familial hypertriglyceridemia is primarily associated with a deficiency of post-heparin-activated lipoprotein lipase. This enzyme plays a crucial role in the hydrolysis of triglycerides in lipoproteins, particularly chylomicrons and very low-density lipoproteins (VLDL). When lipoprotein lipase activity is inadequate, triglycerides accumulate in the bloodstream, leading to elevated levels of triglycerides, which is a hallmark of familial hypertriglyceridemia.

This condition is often genetically inherited and may present with elevated triglyceride levels without significant cholesterol elevation. Understanding the role of lipoprotein lipase is essential, as its deficiency directly impacts lipid metabolism, resulting in the phenotypic expression of high triglyceride levels and associated risks for pancreatitis and cardiovascular disease.

The other options refer to different enzymatic deficiencies or apolipoproteins that are linked to various lipid disorders but do not specifically cause familial hypertriglyceridemia. For example, β-glucocerebrosidase deficiency is associated with Gaucher disease, while Apo-B deficiency relates to problems with the transport of lipoproteins, and Apo-C-III deficiency is linked to enhanced lipolysis but does not specifically cause hypertr