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贫血和红细胞增多症患者血清转铁蛋白受体的定量与特征分析

Quantitation and characterization of serum transferrin receptor in patients with anemias and polycythemias.

作者信息

Kohgo Y, Niitsu Y, Nishisato T, Kato J, Kondo H, Sasaki K, Urushizaki I

机构信息

Department of Internal Medicine, Sapporo Medical College, Japan.

出版信息

Jpn J Med. 1988 Feb;27(1):64-70. doi: 10.2169/internalmedicine1962.27.64.

Abstract

Serum transferrin receptor concentration in patients with anemias and polycythemias was determined. The mean normal value was 258 +/- 82 ng/ml. Comparing with normal values, the higher values were obtained in patients with iron deficiency anemia, autoimmune hemolytic anemia, and polycythemias, while the lower values were obtained in patients with aplsatic anemia. During treatments of patients with iron deficiency anemia and autoimmune hemolytic anemia, serial change of serum receptor was paralleled with that of peripheral reticulocyte counts, suggesting that the circulating receptor values may reflect the activity of bone marrow erythropoiesis. The immunoreactive receptor was migrated at the position of alpha 1-globulin by agarose gel electrophoresis and the isoelectric point(pl) was 3.57 by isoelectric focusing. SDS-polyacrylamide gel electrophorogram of various patients' sera revealed that the autoradiographed band was migrated at the molecular weight of 110,000 daltons in the non-reducing condition and 46,000 and 23,000 daltons in the reducing condition. These bands were also capable of binding to 131I-labelled diferric transferrin. The proposed model of circulating transferrin receptor may be the nicked dimers of 55,000 daltons in which inter- and intra-disulfide bridges were present.

摘要

测定了贫血和红细胞增多症患者的血清转铁蛋白受体浓度。正常均值为258±82 ng/ml。与正常值相比,缺铁性贫血、自身免疫性溶血性贫血和红细胞增多症患者的值较高,而再生障碍性贫血患者的值较低。在缺铁性贫血和自身免疫性溶血性贫血患者的治疗过程中,血清受体的系列变化与外周网织红细胞计数的变化平行,提示循环受体值可能反映骨髓红细胞生成的活性。免疫反应性受体在琼脂糖凝胶电泳中迁移至α1球蛋白位置,等电聚焦法测得其等电点(pl)为3.57。不同患者血清的SDS-聚丙烯酰胺凝胶电泳图谱显示,在非还原条件下,放射自显影片带迁移至分子量110,000道尔顿处,在还原条件下迁移至46,000和23,000道尔顿处。这些条带也能与131I标记的双铁转铁蛋白结合。所提出的循环转铁蛋白受体模型可能是具有分子内和分子间二硫键的55,000道尔顿的带切口二聚体。

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