Use of Plasma Lipoprotein Lipase Activity without Heparin Injection for Detection of Homozygous and Heterozygous Lipoprotein Lipase Deficiency
Use of Plasma Lipoprotein Lipase Activity without Heparin Injection for Detection of Homozygous and Heterozygous Lipoprotein Lipase Deficiency
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使用不注射肝素的血浆脂蛋白脂肪酶活性来检测纯合和杂合脂蛋白脂肪酶缺陷
DOI：
10.1111/j.1749-6632.1991.tb43751.x
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发表时间：
1991
影响因子：
5.2
通讯作者：
C. Corder
中科院分区：
文献类型：
--
作者：
Q. Cheng;P. Blackett;C;J. Fesmire;C. Corder
Following release from an intracellular phosphatidylinositol anchor,'lipoprotein lipase (LPL) is anchored to heparan sulfate on the endothelial cell surface2 in preparation for apo C-I1 activated lipolysis of circulating triglyceride-rich lipoproteins.-'Heparin has traditionally been used to release LPL from the endothelial cell binding sites prior to assay of LPL activity4 but assays have lacked the sensitivity to detect plasma LPL activity without heparin-induced mobilization. Owing to the inconvenience of heparin infusion and the predisposition to hemorrhage, heparin is often contraindicated in children and adults; furthermore depletion of intracellular stores of LPL and continued lipolysis interfere with serial estimates of activity over periods of less than 24 hours. In view of previous use of heparin-Sepharose chromatography for the assay of plasma LPL acti~ ity,~ we developed our own modification based on previous e~ perience.~ Comparison of pre-and postheparin LPL activity showed a correlation (n= 15, p< 0.01)(FIGURE l), whereas hepatic triglyceride lipase did not show any relationship. An infant male proband presented at one month of age with a triglyceride (TG) level of 821 mg/dl. The plasma was noted to be opaque and subsequent examination revealed eruptive xanthomata and lipemia retinalis whereas his twin sister was normal. He had no detectable preheparin LPL activity whereas his parents' activities were low (0.83 and 0.67 nEq/ml per min). Their serum TG, cholesterol, apo AI, apo B, and apo C-I11 were characteristic of a type IV phenotype. Normal values were 5.7 2 2.1 (n= 36). Three adult type V subjects with TG levels over lo00 mg/dl had levels of 1.89 and 1.39, whereas six subjects with TG levels 200 to loo0 mg/dl and four type I diabetic subjects had normal levels (FIGURE 2).
胰岛素通过其磷脂酰肌醇锚的代谢刺激脂蛋白脂肪酶的释放。
DOI：
10.1126/science.241.4873.1670
发表时间：
1988
期刊：
Science (New York, N.Y.)
影响因子：
--
作者：
Chan,BL;Lisanti,MP;Rodriguez-Boulan,E;Saltiel,AR
通讯作者：
Saltiel,AR