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β-地中海贫血/血红蛋白E组织铁蛋白。I:肝脏和脾脏铁蛋白的纯化及部分特性分析。

Beta-thalassaemia/haemoglobin E tissue ferritins. I: Purification and partial characterization of liver and spleen ferritins.

作者信息

Tran K C, Webb J, Macey D J, Pootrakul P, Yansukon P

机构信息

School of Mathematical and Physical Sciences, Murdoch University, Perth, Australia.

出版信息

Biol Met. 1990;3(3-4):222-6. doi: 10.1007/BF01140583.

Abstract

Ferritins from liver and spleen of both beta-thalassaemia/haemoglobin E (HbE) and non-thalassaemic patients were purified by heating a methanol-treated homogenate, followed by molecular exclusion chromatography. The concentrations of ferritins in the beta-thalassaemia/HbE liver and spleen were calculated as 3.8 and 2.0 mg/g wet tissue. The beta-thalassaemia/HbE ferritin iron/protein ratios were higher than those of normal ferritins. On PAGE, all ferritins gave a single major monomeric band with only very small differences in their mobility. Ferritins from thalassaemic patients also possessed bands corresponding to oligomers. On SDS/PAGE, all ferritins were resolved into two major subunits: H and L with L subunit predominating. While the isoferritin profiles of ferritins from beta-thalassaemia/HbE liver and spleen were similar to each other and to those of normal liver and spleen, some extra bands were present in the acidic region. The microstructure of these pathological ferritins appears to result, to a large degree, from the particular nature and amount of iron loading present.

摘要

通过加热经甲醇处理的匀浆,随后进行分子排阻色谱法,从β地中海贫血/血红蛋白E(HbE)患者和非地中海贫血患者的肝脏和脾脏中纯化铁蛋白。β地中海贫血/HbE患者肝脏和脾脏中铁蛋白的浓度经计算分别为3.8和2.0毫克/克湿组织。β地中海贫血/HbE铁蛋白的铁/蛋白比率高于正常铁蛋白。在聚丙烯酰胺凝胶电泳(PAGE)上,所有铁蛋白均呈现出一条单一的主要单体条带,其迁移率仅有非常小的差异。地中海贫血患者的铁蛋白还具有与寡聚体相对应的条带。在十二烷基硫酸钠聚丙烯酰胺凝胶电泳(SDS/PAGE)上,所有铁蛋白均被分离为两个主要亚基:H和L,其中L亚基占主导。虽然β地中海贫血/HbE患者肝脏和脾脏中铁蛋白的铁蛋白异构体谱彼此相似,且与正常肝脏和脾脏的相似,但在酸性区域存在一些额外的条带。这些病理性铁蛋白的微观结构在很大程度上似乎是由所存在的铁负载的特殊性质和量导致的。

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