Cholesteryl Esters

Cholesteryl esters are cholesterol backbones with long-chain fatty acid chains linked to the hydroxyl group. These molecules are much less polar than their non-ester counterparts and are the preferred form for transport in plasma. These molecules do not contribute to the membrane structure but instead are packed into lipid droplets. Cholesteryl esters are found in elevated amounts in adrenal glands and in fatty lesions of atherosclerotic plaques. In plasma and high-density lipoproteins (HDL), cholesteryl esters are synthesized largely by the transfer of fatty acids from the sn-2 position of phosphatidylcholine to cholesterol. Cholesteryl ester storage disease (CESD) is caused by deficient lysosomal acid lipase activity which largely results in cholesteryl ester accumulation in the liver, spleen, and macrophages throughout the body. This disease culminates in microvesicular steatosis leading to liver failure and accelerated atherosclerosis.

Bernstein D., Hulkova, H., Bialer, M., Desnick R. (2013) Cholesteryl ester storage disease: Review of the findings in 135 reported patients with an underdiagnosed disease. J. Hep. 58:1230-1243.