Genetic disorders of lipoprotein metabolism

Lipids, along with proteins and carbohydrates, play a major role in metabolism. They are defined as oils, waxes, fatty acids, and cholesterol. Lipids have a particular role in the physiology of energy metabolism and in the formation of steroid hormones. They are also involved in intracellular signaling, in modulating nerve function, and are responsible for the structural integrity of the cell membrane. In addition, they regulate processes such as protein modification and inflammation. To enable such a diverse range of functions, the pathways of lipid metabolism are closely regulated and also integrated with nonlipid pathways. In order to transport lipids through a predominantly aqueous environment in the body, they are complexed with proteins called apolipoproteins to form macromolecular structures called lipoproteins. The apolipoproteins also function as ligands to enable the interaction between lipoproteins and their receptors and play a role as inhibitors or cofactors for enzymes involved in lipid metabolism.