Glaubensklee C S, Evan A P, Galey W R
Vox Sang. 1982;42(5):262-71. doi: 10.1111/j.1423-0410.1982.tb00755.x.
The abnormal erythrocyte of the McLeod phenotype displays a reduced Kell blood group expression, an abnormal morphology in about 30% of the total red blood cell population and a decrease in water permeability. These parameters have previously been shown to be independent of membrane lipid composition, microviscosity and electrolyte transport. Findings such as these seem to implicate the involvement of a defective membrane protein. The present study investigated membrane composition by utilizing two techniques: freeze-fracture electron microscopy and discontinuous polyacrylamide gel electrophoresis. Electrophoretic scans revealed no apparent band additions or deletions showing McLeod membrane protein composition to be normal. Freeze-fracture electron microscopy did show a significant increase in intramembrane particle density. These findings may be resolved by proposing a dissociation of the band 3 glycoprotein. Unlinkage of this polypeptide, thought to be involved in water transport and intramembranous particle composition, could account for the decreased water transport and increased particle density seen in the McLeod erythrocyte membrane.
麦克劳德表型的异常红细胞表现出凯尔血型表达降低、约30%的总红细胞群体形态异常以及水渗透性降低。这些参数此前已被证明与膜脂质组成、微粘度和电解质转运无关。诸如此类的发现似乎意味着存在有缺陷的膜蛋白。本研究利用两种技术研究了膜组成:冷冻断裂电子显微镜和不连续聚丙烯酰胺凝胶电泳。电泳扫描未显示明显的条带增加或缺失,表明麦克劳德膜蛋白组成正常。冷冻断裂电子显微镜确实显示膜内颗粒密度显著增加。这些发现可以通过提出带3糖蛋白解离来解释。这条被认为参与水运输和膜内颗粒组成的多肽的解偶联,可以解释在麦克劳德红细胞膜中看到的水运输减少和颗粒密度增加的现象。