Utermann G, Kraft H G, Menzel H J, Hopferwieser T, Seitz C
Institut für Medizinische Biologie und Genetik der Universität, Innsbruck, Austria.
Hum Genet. 1988 Jan;78(1):41-6. doi: 10.1007/BF00291232.
The Lp(a) lipoprotein is a complex particle composed of a low density lipoprotein (LDL)-like lipoprotein and the disulfide bonded Lp(a) glycoprotein. The complex represents a quantitative genetic trait. SDS gel electrophoresis under reducing conditions of sera followed by immunoblotting with affinity-purified polyclonal anti-Lp(a) demonstrated inter- and intra-individual size heterogeneity of the glycoprotein with apparent Mr in the range 400-700kDa. According to their relative mobilities compared to apo B-100 the Lp(a) patterns were categorized into phenotypes F, B, S1, S2, S3 und S4 and into the respective double-band phenotypes. This size heterogeneity seems to be controlled by multiple alleles designated LpF, LpB, LpS1, LpS2, LpS3, LpS4 and a null allele (LpO) at a single locus. Phenotype frequencies observed in 441 unrelated subjects were in good agreement with those expected from the genetic hypothesis. Comparison of Lp(a) lipoprotein concentrations in the different phenotypes revealed a highly significant association of phenotypes B, S1 and S2 with high, and phenotypes S3 und S4 with intermediate Lp(a) concentrations. A third mode is represented by the null phenotype were no Lp(a) band is detected upon immunoblotting and Lp(a) lipoprotein is low or absent. We conclude that the same gene locus is involved in determining Lp(a) glycoprotein phenotype and Lp(a) lipoprotein concentrations in plasma. This major gene seems to be the Lp(a) glycoprotein structural gene locus.
脂蛋白(a)[Lp(a)]是一种复合颗粒,由低密度脂蛋白(LDL)样脂蛋白和通过二硫键结合的Lp(a)糖蛋白组成。这种复合物代表一种数量遗传性状。在还原条件下对血清进行十二烷基硫酸钠(SDS)凝胶电泳,随后用亲和纯化的多克隆抗Lp(a)进行免疫印迹,结果显示该糖蛋白在个体间和个体内存在大小异质性,其表观相对分子质量(Mr)在400 - 700 kDa范围内。根据与载脂蛋白B - 100相比的相对迁移率,Lp(a)模式被分为F、B、S1、S2、S3和S4型以及各自的双带型。这种大小异质性似乎由单个基因座上的多个等位基因控制,这些等位基因分别为LpF、LpB、LpS1、LpS2、LpS3、LpS4和一个无效等位基因(LpO)。在441名无亲缘关系的受试者中观察到的表型频率与遗传假设预期的频率高度一致。不同表型中Lp(a)脂蛋白浓度的比较显示,B、S1和S2型与高Lp(a)浓度显著相关,而S3和S4型与中等Lp(a)浓度相关。第三种模式由无效表型代表,在免疫印迹时未检测到Lp(a)条带,且Lp(a)脂蛋白含量低或不存在。我们得出结论,同一基因座参与了血浆中Lp(a)糖蛋白表型和Lp(a)脂蛋白浓度的决定。这个主要基因似乎是Lp(a)糖蛋白结构基因座。