Human lecithin:cholesterol acyltransferase deficiency: in vivo kinetics of low-density lipoprotein and lipoprotein-X.
Human lecithin:cholesterol acyltransferase deficiency: in vivo kinetics of low-density lipoprotein and lipoprotein-X.
复制标题
人卵磷脂：胆固醇酰基转移酶缺乏症：低密度脂蛋白和脂蛋白-X 的体内动力学。
DOI：
10.1161/01.atv.0000217910.90210.99
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发表时间：
2006
期刊：
影响因子：
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通讯作者：
BrewerJr,HBryan
中科院分区：
文献类型：
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作者：
Nishiwaki,Masato;Ikewaki,Katsunori;Bader,Giovanni;Nazih,Hassan;Hannuksela,Minna;Remaley,AlanT;Shamburek,RobertD;BrewerJr,HBryan
目的-卵磷脂：胆固醇酰基转移酶缺乏症（LCAT-def）的特征是高密度脂蛋白（HDL）和低密度脂蛋白（LDL）水平低以及脂蛋白X（LpX）的积累。尽管低密度脂蛋白，动脉粥样硬化是不常见的LCAT-def。降低低密度脂蛋白可能是一个可能的解释，但其潜在的机制尚不清楚。此外，LpX蓄积的机制尚不清楚。本研究的目的是阐明的机制（S）负责低LDL和确定LCAT-def的血浆动力学LpX。方法和结果-我们进行了放射性示踪剂研究LCAT-def（n=2）和正常对照组（n=10）和一个病人和其他控制（n=7）的稳定同位素研究。在对照组和LCAT-def患者中，LCAT-def LDL的分解代谢比对照LDL快。对照LDL在LCAT-def患者中的分解代谢速度比对照快。LDL B-100的生成率在LCAT-def中是正常的。LDL apoB-100催化剂的增加被稳定同位素研究证实。LpX的分解代谢更慢，在LCAT-def。结论LCAT-def中LDL的减少是由于异常LDL的快速catenation和LDL受体途径的上调引起的catenation增加。LpX的催化活性降低有助于其在LCAT-def中的积累。
Objectives—Lecithin:cholesterol acyltransferase deficiency (LCAT-def) is characterized by low levels of high-density lipoprotein (HDL) and low-density lipoprotein (LDL) and the accumulation of lipoprotein-X (LpX). Despite the low HDL, atherosclerosis is uncommon in LCAT-def. The decreased LDL would be a possible explanation but the underlying mechanism is not clear. In addition, the mechanism(s) for LpX accumulation is not known. The aim of the present study is to elucidate the mechanism(s) responsible for the low LDL and determine the plasma kinetics of LpX in LCAT-def.Methods and Results—We conducted a radiotracer study in LCAT-def (n=2) and normal controls (n=10) and a stable isotope study in one patient and other controls (n=7). LCAT-def LDL was catabolized faster than control LDL in the control subjects as well as in LCAT-def patients. Control LDL was catabolized faster in LCAT-def patients than the controls. The production rate of LDL apolipoprotein B-100 was normal in LCAT-def. The increased LDL apoB-100 catabolism was confirmed by a stable isotope study. LpX was catabolized more slowly in LCAT-def.Conclusions—The decreased LDL in LCAT-def is attributable to an increased catabolism caused by a rapid catabolism of abnormal LDL and an upregulation of LDL receptor pathway. The decreased catabolism of LpX contributes to its accumulation in LCAT-def.