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林肯公园血红蛋白(抗Lepore)网织红细胞的珠蛋白链合成失衡。

Unbalanced globin chain synthesis by Hb Lincoln Park (anti-Lepore) reticulocytes.

作者信息

Honig G R, Mason R G, Tremaine L M, Vida L N

出版信息

Am J Hematol. 1978;5(4):335-40. doi: 10.1002/ajh.2830050408.

Abstract

Hemoglobin synthesis was studied in vitro in reticulocytes from a patient with the anti-Lepore variant Hb Lincoln Park. Incorporation of L-leucine-3H into the alpha and beta delta chains of Hb Lincoln Park was substantially less than the incorporation into the corresponding globin chains of Hb A, with the rate of synthesis of the beta delta chain being similar to that of the delta chain of Hb A2. Synthesis of the alpha and total non-alpha globin components was unbalanced, with a substantial excess of alpha chain synthesis. These findings help to explain the mild hemolytic disease present in individuals with this hemoglobin variant.

摘要

对一名患有抗Lepore变异型血红蛋白林肯公园的患者的网织红细胞进行了体外血红蛋白合成研究。L-亮氨酸-3H掺入血红蛋白林肯公园的α链和βδ链的量明显少于掺入血红蛋白A相应珠蛋白链的量,βδ链的合成速率与血红蛋白A2的δ链相似。α链和总非α珠蛋白成分的合成不均衡,α链合成大量过剩。这些发现有助于解释携带这种血红蛋白变异体的个体中存在的轻度溶血性疾病。

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