镰状β地中海贫血骨髓和网织红细胞中的珠蛋白链合成

Globin chain synthesis in sickle beta-thalassaemic bone marrow and reticulocytes.

作者信息

Ladas S, Chalevelakis G, Lyberatos C, Vaidakis E, Arapakis G

出版信息

J Med Genet. 1979 Aug;16(4):296-301. doi: 10.1136/jmg.16.4.296.

DOI:10.1136/jmg.16.4.296

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1012673/

Abstract

Globin chain synthesis was studied in reticulocytes and bone marrow erythroid precursors in four sickle beta-thalassaemic Greek patients. Significant globin chain imbalance was found in reticulocytes (alpha/gamma + beta A + beta S = 2.20 +/- SD 0.16) and bone marrow (alpha/gamma + beta A + beta S = 1.58 +/- SD 0.11) after two hours' incubation. There was evidence of contamination of the gamma, beta A, and, to a lesser extent, of the beta S chain by non-haem proteins. The contamination was more obvious in chromatograms obtained from whole cell bone marrow samples and could partially explain the lower alpha/non-alpha ratio found in bone marrow.

摘要

对4名希腊镰状β地中海贫血患者的网织红细胞和骨髓红细胞前体细胞中的珠蛋白链合成进行了研究。孵育两小时后，在网织红细胞（α/γ + βA + βS = 2.20 ±标准差0.16）和骨髓（α/γ + βA + βS = 1.58 ±标准差0.11）中发现了明显的珠蛋白链失衡。有证据表明，γ、βA链，以及程度较轻的βS链受到了非血红素蛋白的污染。这种污染在从全细胞骨髓样本获得的色谱图中更为明显，并且可以部分解释在骨髓中发现的较低的α/非α比率。

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引用本文的文献

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本文引用的文献

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Heterozygous Beta thalassemia: balanced globin synthesis in bone marrow cells.β 珠蛋白生成障碍性杂合子：骨髓细胞中珠蛋白的平衡合成。

Science. 1970 Mar 13;167(3924):1513-4. doi: 10.1126/science.167.3924.1513.

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THE HEMOGLOBINS.血红蛋白

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Abnormal human haemoglobins. Separation and characterization of the alpha and beta chains by chromatography, and the determination of two new variants, hb Chesapeak and hb J (Bangkok).异常人类血红蛋白。通过色谱法分离和鉴定α链和β链，并测定两种新变体，即血红蛋白切萨皮克和血红蛋白J（曼谷）。

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Asymmetrical incorporation of amino acids in the alpha and beta chains of hemoglobin synthesized by thalassemic reticulocytes.地中海贫血网织红细胞合成的血红蛋白α链和β链中氨基酸的不对称掺入。

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J Mol Biol. 1969 May 28;42(1):57-64. doi: 10.1016/0022-2836(69)90486-0.

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Hemoglobin synthesis during erythroid cell development in thalassemia and other hemolytic anemias.地中海贫血和其他溶血性贫血中红系细胞发育过程中的血红蛋白合成。

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Blood. 1972 Jul;40(1):105-11.

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