title: Small polydisperse low density lipoproteins in familial hyperalphalipoproteinemia with complete deficiency of cholesteryl ester transfer activity.
abstract: Lipoprotein abnormalities were analyzed in 3 cases of marked hyperalphalipoproteinemia caused by complete deficiency of cholesteryl ester transfer activity. The probands were all men, aged 34, 43 and 48 years, respectively. The serum high density lipoprotein (HDL)-cholesterol levels of these patients were higher than 150 mg/dl (157-254 mg/dl), while serum total cholesterol levels ranged from 227 to 360 mg/dl. Sequential flotation-ultracentrifugation analysis disclosed that low density lipoprotein (LDL)-cholesterol was slightly decreased and that cholesteryl ester accumulated solely in the HDL2 fraction, which was also enriched with apolipoprotein E. Cholesteryl ester transfer activity was completely absent in all of these cases. High-performance liquid chromatography showed a decrease of LDL particle size in combination with a marked enlargement of HDL particle size. Analytical ultracentrifugation disclosed heterogeneity of LDL with the presence of small LDL subpopulations. We conclude that hyperalphalipoproteinemia due to complete deficiency of cholesteryl ester transfer activity is characterized by the presence of both small polydisperse LDL and markedly large HDL enriched with cholesteryl ester and apolipoprotein E.
authors: Second Department of Internal Medicine, Osaka University Medical School, Japan.
authors: S
authors: S
authors: Yamashita
authors: Y
authors: Y
authors: Matsuzawa
authors: M
authors: M
authors: Okazaki
authors: H
authors: H
authors: Kako
authors: T
authors: T
authors: Yasugi
authors: H
authors: H
authors: Akioka
authors: K
authors: K
authors: Hirano
authors: S
authors: S
authors: Tarui
mesh_terms: D000328:Adult; D001053:Apolipoproteins / Q000097:blood; D002352:Carrier Proteins / Q000097:blood / Q000172:deficiency*; D002784:Cholesterol / Q000097:blood; D053480:Cholesterol Ester Transfer Proteins; D008078:Cholesterol, LDL / Q000097:blood; D006023:Glycoproteins; D006801:Humans; D006951:Hyperlipoproteinemias / Q000097:blood* / Q000235:genetics; D008077:Lipoproteins, LDL / Q000097:blood*; D008297:Male; D008875:Middle Aged; D014461:Ultracentrifugation
chemical_list: D001053:Apolipoproteins; C506642:CETP protein, human; D002352:Carrier Proteins; D053480:Cholesterol Ester Transfer Proteins; D008078:Cholesterol, LDL; D006023:Glycoproteins; D008077:Lipoproteins, LDL; D002784:Cholesterol