APOB KINETICS IN HETEROZYGOUS LPL DEFICIENCY

有人提出，部分缺陷的受试者 脂蛋白脂酶(LPL)更容易发生冠状动脉早发 动脉疾病是因为他们易合并表达的 脂蛋白表型。为了阐明减少的代谢后果 LPL功能、apoB-100(低密度脂蛋白、低密度脂蛋白和低密度脂蛋白)的产生和分解代谢 极低密度脂蛋白)和apoB48(乳糜粒/残留物)将由 15例脂蛋白脂蛋白缺乏症患者的内源性标记--氚亮氨酸技术 杂合子。13C-亮氨酸推注将同时用于 明确通过率。这些结果将与历史上的 对照组(n=22)。使用相同方法的试点研究将在 3、I型，LPL缺乏者。他们将被不断地研究 平均每个美国人在饮食稳定后处于美联储状态 在4周内节食。脂蛋白组成，包括脂类和 将测定载脂蛋白(SDS-PAGE和ELISA法)。 脂蛋白颗粒大小采用梯度凝胶法测定 电泳法。通过执行这些分析，我们希望解决 以下问题： 1.低密度脂蛋白从极低密度脂蛋白到低密度脂蛋白的加工是否存在缺陷 受试者？ 2.LPL缺陷杂合子apoB100的产生是否增加 与正常对照组相比？ 3.脂蛋白缺乏受试者apoB48分解代谢是否减弱？

It has been proposed that subjects with a partial deficiency of lipoprotein lipase (LpL) are more likely to develop premature coronary artery disease because they are predisposed to the expression of combined lipoprotein phenotypes. To elucidate the metabolic consequences of reduced LpL function, the production and catabolic rates of apoB-100 (LDL, IDL and VLDL), and apoB48 (chylomicrons/remnants) will be determined by the endogenous labeling, deuterated leucine technique in 15 LPL deficient heterozygotes. Bolus 13C-leucine will be administered simultaneously to clarify clearance rates. These results will be compared to historical controls (n=22). A pilot study using the same method will be performed on 3 Type I, LpL deficient subjects. They will be studied in the continuously fed state after dietary stabilization is achieved on an average American diet over a 4 week period. Lipoprotein composition including lipids and apolipoproteins (SDS-PAGE and ELISA assays) will be determined. Lipoprotein particle size will be determined by gradient gel electrophoresis. By performing these analyses, we hope to address the following issues: 1. Is the processing of VLDL to IDL to LDL compromised in LpL deficient subjects? 2. Is the production of apoB100 increased in LpL deficient heterozygotes compared to normal controls? 3. Does apoB48 catabolism diminish in LpL deficient subjects?