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遗传性小细胞贫血中的红细胞铁摄取

Red cell iron uptake in hereditary microcytic anemia.

作者信息

Edwards J A, Hoke J E

出版信息

Blood. 1975 Sep;46(3):381-8.

PMID:807277
Abstract

The iron uptake in vitro of red cells from mice with hereditary microcytic anemia (gene symbol mk) was studied to examine the hypothesis of a generalized impairment of cellular iron uptake in this conidition. Reticulocyte-rich red cells from anemic (mk/mk) and acutely bled normal (+/+) mice were incubated in 59Fe-labeled mouse plasma and the radioiron uptake measured. The 59Fe uptake of the mk/mk and +/+ cells was related in the same way to the reticulocyte concentration, the duration of incubation, and the percentage saturation of the plasma iron-binding capacity. However, under the same conditions, the iron uptake of red cells from normal (+/+) mice was greater than that by red cells from anemic (mk/mk) mice. Furthermore, the cellular loss of radioiron on exposure to EDTA was greater for the mk/mk red cells, although the proportion of the radioiron taken up that was incorporated into heme was the same for mk/mk and +/+ red cells. These results support the hypothesis of a generalized impairment of cellular iron uptake in hereditary microcytic anemia and suggest that there might be a defect in red cell receptor sites for transferrin in this condition.

摘要

研究了遗传性小细胞性贫血(基因符号mk)小鼠红细胞的体外铁摄取,以检验在这种情况下细胞铁摄取普遍受损的假说。将来自贫血(mk/mk)和急性失血正常(+/+)小鼠的富含网织红细胞的红细胞在59Fe标记的小鼠血浆中孵育,并测量放射性铁摄取。mk/mk和+/+细胞的59Fe摄取与网织红细胞浓度、孵育持续时间以及血浆铁结合能力的饱和百分比以相同方式相关。然而,在相同条件下,正常(+/+)小鼠红细胞的铁摄取大于贫血(mk/mk)小鼠红细胞的铁摄取。此外,尽管mk/mk和+/+红细胞摄取的放射性铁掺入血红素的比例相同,但mk/mk红细胞暴露于EDTA时放射性铁的细胞损失更大。这些结果支持遗传性小细胞性贫血中细胞铁摄取普遍受损的假说,并表明在这种情况下转铁蛋白的红细胞受体位点可能存在缺陷。

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