Pagnan A, Zanetti G, Braggion M, Ziron L, Lusiani L, Visona A, Castellani V, Ronsisvalle G
Diabete Metab. 1985 Dec;11(6):343-9.
A new dyslipoproteinemic state, characterized by two populations of very low density lipoprotein particles giving the electrophoretic appearance of two pre-beta bands, the double pre-beta lipoproteinemia, is described. Based upon the lipid and apoprotein composition, it can be inferred that the slow moving pre-beta component is probably made up of remnant very low density lipoprotein particles; the absence of apo B 48 in the very low density lipoprotein fraction in these subjects rules out the intestinal origin of this remnant. Clinical interest in the double pre-beta lipoproteinemia relates to its potential atherogenicity; in fact it seems to be causally associated with such pathological conditions as hypothyroidism, uremia and dialysis, which are frequently accompanied by clinical complications of atherosclerosis. Double pre-beta lipoproteinemia also shows familial aggregation. Six years follow-up in a large family kindred has demonstrated that the family members carrying the double pre-beta lipoproteinemia, develop an incidence of coronary and cerebrovascular events higher than that of family members without the double pre-beta lipoproteinemia.
本文描述了一种新的血脂蛋白异常状态,其特征为两类极低密度脂蛋白颗粒在电泳时呈现出两条前β带,即双前β脂蛋白血症。根据脂质和载脂蛋白的组成可以推断,迁移较慢的前β成分可能由极低密度脂蛋白残余颗粒组成;这些受试者极低密度脂蛋白组分中缺乏载脂蛋白B 48,排除了该残余颗粒的肠道来源。双前β脂蛋白血症的临床意义在于其潜在的致动脉粥样硬化性;事实上,它似乎与甲状腺功能减退、尿毒症和透析等病理状况存在因果关联,而这些状况常伴有动脉粥样硬化的临床并发症。双前β脂蛋白血症也表现出家族聚集性。对一个大家族进行的六年随访表明,携带双前β脂蛋白血症的家庭成员发生冠状动脉和脑血管事件的发生率高于未患双前β脂蛋白血症的家庭成员。