Notario A, Meduri D, Mangiarotti E, Zocchi M T, Azzarà A, Vercesi M
Arch Sci Med (Torino). 1982 Jan-Mar;139(1):9-18.
Total proteins and red cell membrane spectrin were determined in normal subjects, patients with hyperhaemolytic and anhaemolytic thalassaemia, and Hb Lepore from a single family. Electrophoresis on acrylamide gels was performed after solubilisation of the material in SDS using the whole membrane and spectrin. Amino acid composition was also determined after hot acid hydrolysis. Resin chromatography was employed to recognise acid, neutral and basic aminoo acids, glucosamine, and galactosamine. It was found that the significant changes in spectrin amino acid composition observed in thalassaemic subjects with peripheral hyperhaemolysis were not apparent in anhaemolytic patients, nor in the heterozygote, clinically asymptomatic carriers of Hb Lepore. These changes are certainly of importance on account of the structural alterations noted in the spectrin of the subjects concerned.
对正常受试者、高溶血性和无溶血性地中海贫血患者以及来自单个家族的血红蛋白Lepore患者测定了总蛋白和红细胞膜血影蛋白。将材料在SDS中溶解后，使用全细胞膜和血影蛋白在丙烯酰胺凝胶上进行电泳。热酸水解后还测定了氨基酸组成。采用树脂色谱法识别酸性、中性和碱性氨基酸、氨基葡萄糖和半乳糖胺。结果发现，在外周血高溶血性地中海贫血患者中观察到的血影蛋白氨基酸组成的显著变化，在无溶血性患者以及血红蛋白Lepore的临床无症状杂合子携带者中并不明显。鉴于相关受试者血影蛋白中 noted的结构改变，这些变化肯定具有重要意义。
