Evidence for deficiency of high density lipoprotein lecithin: cholesterol acyltransferase activity (alpha-LCAT) in fish eye disease.
Evidence for deficiency of high density lipoprotein lecithin: cholesterol acyltransferase activity (alpha-LCAT) in fish eye disease.
复制标题
高密度脂蛋白卵磷脂缺乏的证据：鱼眼病中胆固醇酰基转移酶活性（α-LCAT）。
作者：
L. Carlson;L. Holmquist
在一种罕见的家族性疾病--鱼眼病中，血浆高密度脂蛋白(HDL)中胆固醇酯的相对含量较低，而极低密度脂蛋白(VLDL)和低密度脂蛋白(LDL)中这些脂类的含量正常。卵磷脂：胆固醇酰基转移酶(LCAT)是介导血浆脂蛋白中游离胆固醇酯化的酶。在目前的研究中，从我们的两个鱼眼病患者分离的高密度脂蛋白被发现是与正常LCAT体外孵育的良好底物，因为存在于对照组的脂蛋白耗竭血浆中。这些高密度脂蛋白组分中几乎所有的游离胆固醇都被酯化，随之而来的是异常小的鱼眼病高密度脂蛋白颗粒增大到正常高密度脂蛋白颗粒的大小。然而，鱼眼病患者的血浆中的脂蛋白耗尽，缺乏正常血浆的性质来酯化从鱼眼病患者或对照组血浆中分离出来的高密度脂蛋白的游离胆固醇。这些结果导致了一个新的概念的形成，暗示在正常血浆中存在两种不同的LCAT活性。其中一种活性称为α-LCAT，专用于高密度脂蛋白(α-脂蛋白)，另一种称为β-LCAT，专用于极低密度脂蛋白(前β和β-脂蛋白)。根据这一概念，鱼眼病被归类为α-LCAT缺乏症，而典型的LCAT缺乏症可能既缺乏α-LCAT活性，也缺乏β-LCAT活性。
In a rare familial condition, fish eye disease, there is a low relative content of cholesteryl esters in the plasma high density lipoproteins (HDL) but a normal content of these lipids in the very low (VLDL) and low (LDL) density lipoproteins. Lecithin: cholesterol acyltransferase (LCAT) is the enzyme which mediates the esterification of free cholesterol in the plasma lipoproteins. In the present investigation, isolated HDL from our two fish eye disease patients were found to be excellent substrates during in vitro incubations with normal LCAT as present in lipoprotein depleted plasma from control subjects. Almost all free cholesterol of these HDL fractions became esterified and concomitantly the abnormally small fish eye disease HDL particles increased to a size in the range of that of normal HDL particles. Lipoprotein depleted plasma from fish eye disease, however, lacked the property of normal plasma to esterify the free cholesterol of HDL isolated from plasma of fish eye disease patients or control subjects. These results have led to the formulation of a new concept implying that two different LCAT activities exist in normal plasma. One of these activities, denoted alpha-LCAT, is specific for HDL (alpha-lipoproteins) and the other, beta-LCAT, is specific for VLDL-LDL (pre beta- and beta-lipoproteins). Fish eye disease according to this notion is classified as an alpha-LCAT deficiency in contrast to the classical LCAT deficiency which probably lacks both alpha- and beta-LCAT activities.
脂蛋白受体胆固醇酯含量调节人血浆卵磷脂：胆固醇酰基转移酶活性。
DOI：
--
发表时间：
1981
期刊：
The Journal of biological chemistry
影响因子：
--
作者：
Fielding,CJ;Fielding,PE
通讯作者：
Fielding,PE