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血红蛋白F中链合成的不平衡。

The imbalance of chain synthesis in hemoglobin F.

作者信息

Rogers C, Kay L M, Schroeder W A, Powars D

出版信息

Hemoglobin. 1981;5(6):531-48. doi: 10.3109/03630268108991685.

Abstract

The synthesis of Hb F has been found to be unbalanced in a number of conditions, including homozygous HPFH, sickle-cell anemia, and normal term cord bloods. In this study, the counts from the pre-gamma and pre-alpha zones of the chain separation chromatogram are considered to be modified gamma and alpha chains, respectively, and are included in calculating the gamm/alpha ratios for cord blood and sickle-cell blood. The gamma chain is labeled about 52% as efficiently as the alpha chain in reticulocytes. It is possible that nu chain synthesis is balanced in the bone marrow, but subsequently becomes unbalanced in the reticulocyte.

摘要

已发现在多种情况下,包括纯合子遗传性胎儿血红蛋白持续存在症(HPFH)、镰状细胞贫血和足月正常脐带血中,血红蛋白F(Hb F)的合成是不平衡的。在本研究中,链分离色谱图的前γ区和前α区的计数分别被视为修饰的γ链和α链,并包含在计算脐带血和镰状细胞血的γ/α比率中。在网织红细胞中,γ链的标记效率约为α链的52%。有可能在骨髓中ν链的合成是平衡的,但随后在网织红细胞中变得不平衡。

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