Schlüter K, Drenckhahn D
Proc Natl Acad Sci U S A. 1986 Aug;83(16):6137-41. doi: 10.1073/pnas.83.16.6137.
Precipitates of hemoglobin, termed Heinz bodies, occur in a fraction of erythrocytes after removal of the spleen and are also observed in aged erythrocytes. This implies that precipitates of hemoglobin might play a particular role in senescent cell recognition. By using immunofluorescence microscopy, evidence is presented in splenectomized patients and in several patients with unstable (mutant) hemoglobins that membrane-attached Heinz bodies are associated with both clusters of the anion channel, band 3, and clusters of surface-bound immunoglobulins (IgG). In 75% of the cases of unstable hemoglobin, such as sickle cell anemia or hemoglobin Köln disease, the level of cell-bound IgG (measured by 125I-labeled staphylococcal protein A) was increased severalfold above the level found in healthy controls. Immunoblot analysis identified the major fraction of cell-bound IgG to be directed to band 3. These observations indicate copolymerization of denatured hemoglobin with the cytoplasmic domain of band 3, which may cause band 3 to form clusters. These clusters probably serve as thermodynamically favored binding sites for autoantibodies in serum, which promote elimination of the erythrocytes by the immune system. Thus, erythrocytes may be removed from circulation when hemoglobin begins to denature and the cells begin to fail in their main function of oxygen transport.
被称为海因茨小体的血红蛋白沉淀物，在脾脏切除后的一部分红细胞中出现，在衰老红细胞中也可观察到。这意味着血红蛋白沉淀物可能在衰老细胞识别中发挥特殊作用。通过免疫荧光显微镜检查，在脾切除患者和几名患有不稳定（突变）血红蛋白的患者中发现，附着在细胞膜上的海因茨小体与阴离子通道带3的簇以及表面结合的免疫球蛋白（IgG）簇有关。在75%的不稳定血红蛋白病例中，如镰状细胞贫血或血红蛋白科隆病，细胞结合IgG的水平（通过125I标记的葡萄球菌蛋白A测量）比健康对照者高出几倍。免疫印迹分析确定细胞结合IgG的主要部分是针对带3的。这些观察结果表明变性血红蛋白与带3的细胞质结构域发生共聚，这可能导致带3形成簇。这些簇可能作为血清中自身抗体在热力学上有利的结合位点，促进免疫系统清除红细胞。因此，当血红蛋白开始变性且细胞开始无法发挥其主要的氧气运输功能时，红细胞可能会从循环中被清除。
