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镰状细胞贫血和β⁰地中海贫血患者骨髓细胞中的珠蛋白合成:β链被非珠蛋白污染。

Globin synthesis in bone marrow cells of patients with sickle cell anemia and beta O-thalassemia: contamination of the beta-chain with non-globin proteins.

作者信息

Cividalli G, Kerem H, Rachmilewitz E A

出版信息

Hemoglobin. 1979;3(2-3):175-83. doi: 10.3109/03630267908998912.

Abstract

Chromatographic separation of labeled globin chains was performed in stroma-free hemolysates prepared from peripheral blood and bone marrow cells of 11 patients with beta O-thalassemia and 2 patients with sickle cell anemia. A small radioactivity peak, slightly preceding the beta-chain and more prominent in bone marrow cells, was often observed. This peak, which represents synthesis of non-globin proteins, did not exceed 5% of the radioactivity incorporated in the alpha-chain. It is concluded that contamination of the beta-chain with non-globin proteins undoubtedly occurs, but its extent is insufficient to explain the different synthetic ratios which have been repeatedly observed in peripheral blood and in bone marrow cells of patients with heterozygous beta-thalassemia.

摘要

对11例β⁰地中海贫血患者和2例镰状细胞贫血患者的外周血及骨髓细胞制备的无基质溶血产物进行了标记珠蛋白链的色谱分离。经常观察到一个小的放射性峰,略先于β链,在骨髓细胞中更明显。这个代表非珠蛋白合成的峰,其放射性不超过掺入α链的5%。得出的结论是,β链无疑会被非珠蛋白污染,但其程度不足以解释在杂合子β地中海贫血患者外周血和骨髓细胞中反复观察到的不同合成比例。

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