Studies on the Preventive System Against Atherosclerosis Based on the Discovery of Cases With Choles-Terol Ester Transfer Protein Deficiency.

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High density lipoprotein has been believed to play an important role in protecting atherosclerosis and the deficiency of HDL causes premature atherosclerosis. However, we have reported some types of hyper HDL cholesterolemia may be accompanied with lipid storage including coronary atherosclerosis and corneal opacities similar to HDL deficiency. In this study, we found the cases with hyper HDL cholesterolemia with Chdesterol Ester Transfer Protein (CETP) deficiency and studied the lipoprotein metabolism in this metabolic disorder to clarify physiological roles of CETP in the preventive system against atherosclerosis. HDL pasticles were shown to be markidly enlarged with high content of cholesteryl ester (CE) and apolipoprotein E. We demonstrated that LDL penticles also became abnormal in CETP deficiency. Namely, particles became polydisperse and consist of two groups different in size. These result suggests that CETP may play an important role in transferring CE from HDL to LDL and forming mature and homogeneous LDL.The defect of this process may result in cholesterol transport system from peripheral tissues to liver and finally cause the impairment of prevention against lipid storage in tissues.