Raum D, Spence M A, Balavitch D, Tideman S, Merritt A D, Taggart R T, Petersen B H, Day N K, Alper C A
J Clin Invest. 1979 Sep;64(3):858-65. doi: 10.1172/JCI109534.
Using isoelectric focusing in polyacrylamide gel and a hemolytic assay for development of patterns, extensive, structural polymorphism in human C8 has been delineated. Two alleles, C8A and C8B, have been identified in orientals, with gene frequencies of 0.655 and 0.345. In blacks, what appears to be a third common allele was found, so that frequencies were 0.692, 0.259, and 0.049 for C8A, C8B, and C8A1. In whites, C8A1 was rare with a frequency of 0.003, and frequencies for C8A and C8B were 0.649 and 0.349. Inheritance was autosomal codominant in family studies and the distribution of types in random unrelated populations fit the Hardy-Weinberg equilibrium in all groups. C8 allotypes have been determined for two previously studied families, each with a homozygous C8-deficient propositus. This study suggests that C8 deficiency is a silent or null allele of the C8 structural locus, and that half normal levels of C8 cannot be used as a single criterion for the establishment of heterozygous C8 deficiency. C8 allotypes, as well as 18 other autosomal markers, were also determined for 24 families. The C8 structural locus is not closely linked to these markers, including the human histocompatibility loci complex.
利用聚丙烯酰胺凝胶等电聚焦和溶血试验来展现图谱,已描绘出人类补体C8广泛的结构多态性。在东方人中已鉴定出两个等位基因,即C8A和C8B,基因频率分别为0.655和0.345。在黑人中,发现了似乎是第三个常见等位基因,因此C8A、C8B和C8A1的频率分别为0.692、0.259和0.049。在白人中,C8A1罕见,频率为0.003,C8A和C8B的频率分别为0.649和0.349。家系研究表明其遗传方式为常染色体共显性,并且在所有群体中,随机无关人群中各类型的分布均符合哈迪-温伯格平衡。已确定了两个先前研究过的家系的C8同种异型,每个家系都有一个纯合C8缺陷的先证者。这项研究表明,C8缺陷是C8结构基因座的一个沉默或无效等位基因,并且不能将C8正常水平的一半作为确立杂合性C8缺陷的单一标准。还为24个家系确定了C8同种异型以及其他18个常染色体标记。C8结构基因座与这些标记,包括人类组织相容性基因座复合体,没有紧密连锁。