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β地中海贫血/血红蛋白E组织铁蛋白。II:心脏和胰腺铁蛋白与肝脏和脾脏铁蛋白的比较。

Beta-thalassaemia/haemoglobin E tissue ferritins. II: A comparison of heart and pancreas ferritins with those of liver and spleen.

作者信息

Tran K C, Webb J, Macey D J, Pootrakul 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):227-31. doi: 10.1007/BF01140584.

Abstract

Tissue ferritins from beta-thalassaemia/haemoglobin E heart and pancreas were characterized by native PAGE, SDS/PAGE and isoelectric focussing, and compared with those isolated from corresponding liver and spleen tissue. On PAGE, all ferritins consisted of a single band assigned to the protein monomer. Small differences in electrophoretic mobility were found between the bands. The ferritins were resolved by SDS/PAGE into two major subunits, H and L, corresponding to molecular masses of 22.5 kDa and 19 kDa, respectively. The L subunit was predominant in all cases. The isoferritin profiles of all tissue ferritins were remarkably similar, consisting of a complex pattern of bands which were appreciably more basic than those obtained for horse spleen ferritin. The subunit composition and isoferritin profiles of the four tissue ferritins almost certainly reflect the defense mechanism of the body in synthesizing in all four tissue types a more stable long-term iron-storage isoferritin in order to detoxify and store the excess iron present due to the pathological condition of beta-thalassaemia/HbE.

摘要

通过非变性聚丙烯酰胺凝胶电泳(native PAGE)、十二烷基硫酸钠-聚丙烯酰胺凝胶电泳(SDS/PAGE)和等电聚焦对β地中海贫血/血红蛋白E患者心脏和胰腺的组织铁蛋白进行了表征,并与从相应肝脏和脾脏组织中分离出的铁蛋白进行了比较。在PAGE上,所有铁蛋白均由一条对应于蛋白质单体的条带组成。条带之间的电泳迁移率存在微小差异。铁蛋白通过SDS/PAGE分离为两个主要亚基,H和L,分别对应于分子量22.5 kDa和19 kDa。在所有情况下,L亚基均占主导地位。所有组织铁蛋白的异铁蛋白谱非常相似,由一组明显比马脾铁蛋白条带更偏碱性的复杂条带模式组成。这四种组织铁蛋白的亚基组成和异铁蛋白谱几乎可以肯定地反映了身体的防御机制,即在所有四种组织类型中合成一种更稳定的长期铁储存异铁蛋白,以便对由于β地中海贫血/HbE病理状况而存在的过量铁进行解毒和储存。

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