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脱氧镰状红细胞中的纤维、晶体及其他形式的血红蛋白S聚合物。

Fibers, crystals, and other forms of HbS polymers in deoxygenated sickle erythrocytes.

作者信息

Kaperonis A A, Handley D A, Chien S

出版信息

Am J Hematol. 1986 Mar;21(3):269-75. doi: 10.1002/ajh.2830210306.

Abstract

We have examined by electron microscopy the formation of fibers and crystals from sickle hemoglobin within sickle erythrocytes following deoxygenation during capillary storage from 1 to 132 days. Intracellular fibers were found on the first day and throughout the period of study. The fibers exhibited a diameter (mean +/- SD) of 17.4 +/- 0.62 nm and were aligned in the cell with a fiber-to-fiber spacing of 18.6 nm (x-axis) by 22.7 nm (y-axis). Between 65 and 132 days, extracellular hemoglobin crystals developed, with a lattice periodicity of 9.63 +/- 0.6 nm. Fibers and crystals coexist as separate structures. These results suggest that crystal formation upon storage of packed deoxygenated sickle erythrocytes may proceed via a phase of fiber dissolution followed by hemoglobin reassembly into extracellular crystals, rather than by a progressive alignment and direct fusion of existing fibers.

摘要

我们通过电子显微镜检查了镰状红细胞在毛细血管储存1至132天期间脱氧后,镰状血红蛋白在细胞内形成纤维和晶体的情况。在储存第一天及整个研究期间均发现了细胞内纤维。这些纤维的直径(平均值±标准差)为17.4±0.62纳米,在细胞内排列时,纤维间间距在x轴为18.6纳米,y轴为22.7纳米。在65至132天之间,细胞外血红蛋白晶体形成,其晶格周期为9.63±0.6纳米。纤维和晶体作为独立结构共存。这些结果表明,储存的脱氧镰状红细胞在储存时形成晶体可能是通过纤维溶解阶段,随后血红蛋白重新组装成细胞外晶体,而不是通过现有纤维的逐步排列和直接融合。

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