正常胆固醇血症受试者与杂合子家族性高胆固醇血症患者的低密度脂蛋白与肝细胞膜的可饱和结合差异。
Difference in saturable binding of low density lipoprotein to liver membranes from normocholesterolemic subjects and patients with heterozygous familial hypercholesterolemia.
作者信息
Harders-Spengel K, Wood C B, Thompson G R, Myant N B, Soutar A K
出版信息
Proc Natl Acad Sci U S A. 1982 Oct;79(20):6355-9. doi: 10.1073/pnas.79.20.6355.
Abstract
To investigate the possible role of the low density lipoprotein (LDL) receptor in the catabolism of LDL by the human liver, the binding of 125I-labeled LDL to membrane fractions prepared from human liver biopsies was determined. Biopsy samples taken for routine histology were obtained from seven patients with heterozygous familial hypercholesterolemia, one with non-familial hypercholesterolemia, and seven normocholesterolemic subjects. LDL was bound by the membranes from normal subjects in a saturable manner that was inhibited by 56% in the presence of excess LDL. Binding of LDL was also inhibited by modification of the lipoproteins with 1,2-cyclohexanedione. The amount of 125I-labeled LDL bound to membranes from familial hypercholesterolemic livers that could be displaced with excess LDL was significantly less than that bound by normocholesterolemic membranes. These observations suggest that LDL receptors are expressed in normal human liver and are defective in the livers of familial hypercholesterolemic patients.
摘要
为了研究低密度脂蛋白（LDL）受体在人肝脏对LDL分解代谢中的可能作用，测定了125I标记的LDL与人肝脏活检制备的膜组分的结合情况。用于常规组织学检查的活检样本取自7例杂合子家族性高胆固醇血症患者、1例非家族性高胆固醇血症患者和7例胆固醇正常的受试者。正常受试者的膜以可饱和的方式结合LDL，在存在过量LDL的情况下，结合被抑制56%。用1,2 - 环己二酮修饰脂蛋白也可抑制LDL的结合。家族性高胆固醇血症肝脏的膜结合的125I标记LDL，能用过量LDL置换的量明显少于胆固醇正常的膜结合的量。这些观察结果表明，LDL受体在正常人类肝脏中表达，而在家族性高胆固醇血症患者的肝脏中存在缺陷。
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Protein measurement with the Folin phenol reagent.使用福林酚试剂进行蛋白质测定。
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