THE METABOLISM OF VERY LOW-DENSITY AND INTERMEDIATE DENSITY LIPOPROTEINS IN PATIENTS WITH FAMILIAL HYPERCHOLESTEROLEMIA
THE METABOLISM OF VERY LOW-DENSITY AND INTERMEDIATE DENSITY LIPOPROTEINS IN PATIENTS WITH FAMILIAL HYPERCHOLESTEROLEMIA
复制标题
DOI：
10.1016/0021-9150(82)90024-7
复制
发表时间：
1982-01-01
期刊：
影响因子：
5.3
通讯作者：
THOMPSON, GR
中科院分区：
文献类型：
--
作者：
SOUTAR, AK;MYANT, NB;THOMPSON, GR
研究了正常人和家族性高胆固醇血症（FH）患者静脉注射125I标记的自体VLDL后载脂蛋白B （apoB）在极低密度脂蛋白（VLDL）和中密度脂蛋白（IDL）中的代谢。正常受试者、FH杂合子和FH纯合子的VLDL-apoB半衰期、池大小和周转率（mg/kg / h）均无显著差异。FH患者的IDL-apoB代谢与正常人有显著差异。FH患者比活度曲线上升到最大值的速度较正常人慢，比活度曲线下降肢半衰期较长，循环分数率较低，血药浓度高于正常人。在正常受试者中，胆甾胺对idl -载脂蛋白代谢的影响与在FH杂合子和纯合子中没有区别，尽管已知胆甾胺可以刺激LDL受体对肝脏低密度脂蛋白（LDL）的摄取。在正常人中，LDL受体可能对肝脏摄取由VLDL衍生的IDL-载脂蛋白ob有一定贡献，但IDL的摄取部分是由一个单独的受体介导的，该受体识别载脂蛋白E，但不识别载脂蛋白ob。
The metabolism of apolipoprotein B (apoB) in very low density lipoprotein (VLDL) and intermediate density lipoprotein (IDL) was studied in normal subjects and in patients with familial hypercholesterolemia (FH) after an i.v. injection of autologous VLDL labeled with 125I. There were no significant differences in half-life, pool size and turnover rate (mg/kg per h) of VLDL-apoB between the normal subjects, the FH heterozygotes and the FH homozygotes. IDL-apoB metabolism in the FH patients differed significantly from that in the normal subjects. In the FH patients, the rise to the maximum of the specific activity curve was slower, the half-life of the descending limb of the specific-activity curve was longer, the fractional rate of turnover was lower and the plasma concentration was higher than in the normals. The effect of cholestyramine on IDL-apoB metabolism in the normal subjects did not differ from that in the FH heterozygotes and homozygotes, though cholestyramine is known to stimulate hepatic uptake of low density lipoprotein (LDL) by the LDL receptor. In normal human subjects the LDL receptor probably makes some contribution to the hepatic uptake of IDL-apoB derived from VLDL, but IDL uptake is mediated partly by a separate receptor that recognizes apolipoprotein E but not apoB.