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关于Ⅲ型高脂蛋白血症中的脂蛋白异常。

On thelipoprotein abnormality in type 3 hyperlipoproteinemia.

作者信息

Quarfordt S, Levy R I, Fredrickson D S

出版信息

J Clin Invest. 1971 Apr;50(4):754-61. doi: 10.1172/JCI106546.

DOI:10.1172/JCI106546
PMID:5547274
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC291989/
Abstract

Two lipoprotein species were isolated by starch block electrophoresis from the very low density lipoproteins (VLDL) (S(f) 20-400) of patients with type III hyperlipoproteinemia. One of these, alpha(2)-VLDL, had a content of lipid and protein and physical characteristics similar to VLDL from normal subjects or patients with other forms of hyperglyceridemia. The other species, beta-VLDL, contained more cholesterol and less triglyceride in relation to the protein, than normal VLDL. Only the apoprotein of low density lipoprotein was immunochemically detectable in the beta-VLDL; the proteins in the alpha(2)-VLDL reacted with antisera specific for low density lipoprotein and high density lipoprotein. The electrophoretic mobility of beta-VLDL was similar to that of low density lipoprotein and significantly less than that of alpha(2)-VLDL. Isolated beta-VLDL had a lesser mean flotation rate than alpha(2)-VLDL, but both alpha(2)- and beta-VLDL were found throughout the S(f) 20-400 flotation range.The relative quantities of alpha(2)- and beta-VLDL could be varied by changing the diet or by heparin administration. Most of the VLDL from type III patients on a high carbohydrate diet was in the alpha(2)-VLDL form. During fasting, alpha(2)-VLDL fell and beta-VLDL increased becoming the predominant species of VLDL. Heparin-induced acceleration of triglyceride clearance also increased beta-VLDL and decreased alpha(2)-VLDL. These findings suggest a precursor-product relationship between the alpha(2)- and beta-forms of VLDL.

摘要

通过淀粉块电泳从Ⅲ型高脂蛋白血症患者的极低密度脂蛋白(VLDL)(S(f) 20 - 400)中分离出两种脂蛋白。其中一种,α(2)-VLDL,其脂质和蛋白质含量以及物理特性与正常受试者或其他形式高甘油三酯血症患者的VLDL相似。另一种,β-VLDL,与正常VLDL相比,相对于蛋白质含有更多的胆固醇和更少的甘油三酯。在β-VLDL中仅能通过免疫化学方法检测到低密度脂蛋白的载脂蛋白;α(2)-VLDL中的蛋白质与针对低密度脂蛋白和高密度脂蛋白的抗血清发生反应。β-VLDL的电泳迁移率与低密度脂蛋白相似,且明显低于α(2)-VLDL。分离出的β-VLDL的平均漂浮率低于α(2)-VLDL,但α(2)-VLDL和β-VLDL在整个S(f) 20 - 400漂浮范围内均有发现。通过改变饮食或给予肝素可以改变α(2)-VLDL和β-VLDL的相对量。食用高碳水化合物饮食的Ⅲ型患者的大多数VLDL呈α(2)-VLDL形式。禁食期间,α(2)-VLDL减少,β-VLDL增加并成为VLDL的主要形式。肝素诱导的甘油三酯清除加速也会增加β-VLDL并减少α(2)-VLDL。这些发现提示VLDL的α(2)-和β-形式之间存在前体-产物关系。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/05aa/291989/f1ba8c1581bf/jcinvest00193-0064-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/05aa/291989/f1ba8c1581bf/jcinvest00193-0064-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/05aa/291989/f1ba8c1581bf/jcinvest00193-0064-a.jpg

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2
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Klin Wochenschr. 1981 Sep 15;59(18):1023-35. doi: 10.1007/BF01747745.
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本文引用的文献

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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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Interaction between oleate and the lipoproteins of human serum.油酸与人血清脂蛋白之间的相互作用。
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Selective measurement of two lipase activities in postheparin plasma from normal subjects and patients with hyperlipoproteinemia.对正常受试者和高脂蛋白血症患者肝素化后血浆中两种脂肪酶活性的选择性测定。
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Primary dysbetalipoproteinemia: predominance of a specific apoprotein species in triglyceride-rich lipoproteins.原发性异常β脂蛋白血症:富含甘油三酯的脂蛋白中特定载脂蛋白种类占优势。
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Pre-beta-very low density lipoproteins as precursors of beta-very low density lipoproteins. A model for the pathogenesis of familial dysbetalipoproteinemia (type III hyperlipoproteinemia).前β-极低密度脂蛋白作为β-极低密度脂蛋白的前体。家族性异常β脂蛋白血症(III型高脂蛋白血症)发病机制的一种模型。
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