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血色素沉着症中的血清铁蛋白:放血疗法期间异铁蛋白组成的变化

Serum ferritin in haemochromatosis: changes in the isoferritin composition during venesection therapy.

作者信息

Halliday J W, McKeering L V, Tweedale R, Powell L W

出版信息

Br J Haematol. 1977 Jul;36(3):395-404. doi: 10.1111/j.1365-2141.1977.tb00663.x.

Abstract

The isoferritin composition of serum ferritin in 13 patients with untreated idiopathic haemochromatosis (IHC) has been shown to differ from normal in exhibiting an increase in isoferritins in the pH range 5.54-5.62. A similar change was observed in four patients with gross iron overload secondary to haemolytic anaemia. During the course of venesection therapy there was a progressive rise in isoferritins of pI 5.02-5.06 relative to the more basic isoferritins. These observations are consistent with previous studies showing alterations in tissue isoferritins in untreated IHC before and after venesection therapy and they are compatible with the hypothesis that the more basic isoferritins correspond to a 'storage' ferritin and the more acidic to a 'secretory' ferritin. The studies also provide further evidence for a possible biological role of the individual isoferritins.

摘要

13例未经治疗的特发性血色素沉着症(IHC)患者血清铁蛋白的异铁蛋白组成已显示出与正常情况不同,即在pH值5.54 - 5.62范围内异铁蛋白增加。在4例继发于溶血性贫血的严重铁过载患者中也观察到了类似变化。在放血治疗过程中,相对于碱性更强的异铁蛋白,pI为5.02 - 5.06的异铁蛋白逐渐升高。这些观察结果与之前的研究一致,即在放血治疗前后未经治疗的IHC中组织异铁蛋白发生改变,并且与以下假设相符:碱性更强的异铁蛋白对应于“储存”铁蛋白,而酸性更强的对应于“分泌”铁蛋白。这些研究还为单个异铁蛋白可能的生物学作用提供了进一步证据。

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