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为什么家族性高乳糜微粒血症患者的极低密度脂蛋白水平正常。

Why very low density lipoprotein levels are normal in familial hyperchylomicronaemia.

作者信息

Berger G M

出版信息

Atherosclerosis. 1979 Sep;34(1):83-6. doi: 10.1016/0021-9150(79)90109-6.

DOI:10.1016/0021-9150(79)90109-6
PMID:227432
Abstract

The anomalous finding that very low density lipoprotein levels are relatively normal in patients with familial hyperchylomicronaemia has never been satisfactorily explained, particularly in view of the marked reduction or absence of peripheral lipoprotein lipase activity characteristic of this condition. I propose that the discrepancy between the plasma levels of the two triglyceride-rich lipoprotein fractions in these patients is due to the secretion by the liver of triglyceride in the form of chylomicron-like particles, rather than as very low density lipoprotein. The proposed "switch" in the spectrum of lipoproteins secreted by the liver is probably contingent upon the activity of the hepatic lipase present on the liver cell plasma membrane.

摘要

家族性高乳糜微粒血症患者极低密度脂蛋白水平相对正常这一异常发现从未得到令人满意的解释,尤其是考虑到该病症特征性的外周脂蛋白脂肪酶活性显著降低或缺乏。我认为这些患者中两种富含甘油三酯的脂蛋白组分血浆水平之间的差异是由于肝脏以乳糜微粒样颗粒形式分泌甘油三酯,而非以极低密度脂蛋白形式分泌。肝脏分泌的脂蛋白谱中所提出的这种“转换”可能取决于存在于肝细胞膜上的肝脂肪酶的活性。

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Why very low density lipoprotein levels are normal in familial hyperchylomicronaemia.为什么家族性高乳糜微粒血症患者的极低密度脂蛋白水平正常。
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引用本文的文献

1
Chylomicron-like particles in severe hypertriglyceridemia.严重高甘油三酯血症中的乳糜微粒样颗粒。
Lipids. 1985 Apr;20(4):211-5. doi: 10.1007/BF02534190.