Agre P, Smith B L, Hartel-Schenk S
Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland.
Yale J Biol Med. 1990 Sep-Oct;63(5):461-7.
The clinically important Rh blood group system is complex, consisting of multiple distinct antigens. Despite clinical recognition for over 50 years, the Rh blood group antigens have remained poorly understood on a molecular level until the recent identification and characterization of the "Rh polypeptides," the core structural proteins of the Rh antigens. This group of erythrocyte membrane proteins of molecular weight 30,000-35,000 daltons was first recognized by employing Rh-specific antibodies to immunoprecipitate radiolabeled components of erythrocyte membranes. By using antibodies specific for the Rh D, c, and E antigens, a series of highly related non-identical proteins were immunoprecipitated, indicating that the Rh antigens are composed of multiple related proteins. The Rh polypeptides have been purified and characterized, and they were found to have several unusual biochemical characteristics. The Rh polypeptides penetrate the membrane bilayer; they are linked to the underlying membrane skeleton; they are covalently fatty acid acylated with palmitate. While the Rh antigenic reactivity is unique to human erythrocytes, the Rh polypeptides have been isolated from erythrocytes of diverse species and are thought to be fundamental components of all mammalian erythrocyte membranes. The functional role of the Rh polypeptides remains undefined, but a role in the organization of membrane phospholipid is suspected.
具有临床重要性的Rh血型系统很复杂，由多种不同的抗原组成。尽管在临床上已被认识超过50年，但直到最近“Rh多肽”（Rh抗原的核心结构蛋白）被鉴定和表征，Rh血型抗原在分子水平上仍了解甚少。这组分子量为30,000 - 35,000道尔顿的红细胞膜蛋白最初是通过使用Rh特异性抗体免疫沉淀红细胞膜的放射性标记成分而被识别的。通过使用针对Rh D、c和E抗原的特异性抗体，一系列高度相关但不完全相同的蛋白质被免疫沉淀，这表明Rh抗原由多种相关蛋白质组成。Rh多肽已被纯化和表征，发现它们具有几个不同寻常的生化特性。Rh多肽穿透膜双层；它们与下面的膜骨架相连；它们被棕榈酸共价脂肪酸酰化。虽然Rh抗原反应性是人类红细胞所特有的，但Rh多肽已从多种物种的红细胞中分离出来，被认为是所有哺乳动物红细胞膜的基本成分。Rh多肽的功能作用尚不清楚，但怀疑其在膜磷脂组织中起作用。
