desmosterol and Lecithin-Cholesterol-Acyltransferase-Deficiency

Desmosterol and beta-sitosterol were esterified when incubated with normal human plasma. The initial rate of demosterol esterification was 1.7 times faster, and that of beta-sitosterol 0.4 times slower, than the esterification rate of cholesterol. These ratios were found to be almost the same when plasma from different normal individuals was tested. Plasma from a patient with familial lecithin:cholesterol acyltransferase deficiency did not esterify any of the sterols. The esterification of desmosterol and beta-sitosterol was considerably slower in normal plasma in which in vitro cholesterol esterification previously had taken place. The different esterification rates could not be explained by a different affinity of the plasma lipoproteins for the sterols tested. Cholecalciferol added to normal plasma did not become esterified.

Topics: Acyltransferases; Adult; Cholecalciferol; Cholesterol; Desmosterol; Esterification; Female; Humans; Lecithin Cholesterol Acyltransferase Deficiency; Lipids; Lipoproteins; Male; Phosphatidylcholine-Sterol O-Acyltransferase; Sitosterols; Time Factors