Porter R R
Mol Biol Med. 1983 Jul;1(1):161-8.
Genes in the major histocompatibility complex code for three major groups of glycoproteins, now referred to as classes I, II, and III. Susceptibility to some autoimmune diseases and to systemic lupus erythematosus is associated with the presence of particular haplotypes of genes in these three classes. An attempt has been made to correlate these finding on the basis of the observation that different polymorphic forms of complement component C4 show varying efficiencies of complement activation. It is suggested that susceptibility to these diseases will be related to the varying efficiency of complement cell lysis and of immune aggregate dissolution by complement. This in turn will depend on the strength of interaction of the different polymorphic forms of C4 with other proteins (some also polymorphic) in the scheme of activation and inactivation of complement. Such an arrangement would lead to preferential association of certain alleles of C2, C4 and factor B and possibly also of class I and II antigens as potential targets of complement reaction.
主要组织相容性复合体中的基因编码三类主要的糖蛋白,现在称为I类、II类和III类。对某些自身免疫性疾病和系统性红斑狼疮的易感性与这三类基因中特定单倍型的存在有关。基于不同多态形式的补体成分C4表现出不同的补体激活效率这一观察结果,人们试图将这些发现联系起来。有人提出,对这些疾病的易感性将与补体细胞溶解和补体溶解免疫聚集体的不同效率有关。这反过来又取决于C4不同多态形式与补体激活和失活过程中其他蛋白质(有些也是多态的)相互作用的强度。这样的安排将导致C2、C4和B因子的某些等位基因以及可能还有I类和II类抗原作为补体反应的潜在靶点优先关联。