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SS型和AS型红细胞中镰状血红蛋白聚合物的测定。

Determination of sickle hemoglobin polymer in SS and AS erythrocytes.

作者信息

Noguchi C T, Torchia D A, Schechter A N

出版信息

Blood Cells. 1982;8(2):225-35.

PMID:7159747
Abstract

Using 13C nuclear magnetic resonance techniques, we have studied the polymerization of hemoglobin S and intracellular polymerization in SS and AS erythrocytes. For SS erythrocytes, polymer could be detected at high oxygen saturations (greater than 90%) and increased with decreasing oxygen saturation to 70% of the total hemoglobin at complete deoxygenation. These results are consistent with the non-ideal behavior of hemoglobin S at the high intracellular concentration of 34 g/dl. The amount of polymer in AS erythrocytes was also measured as a function of oxygen saturation and is in agreement with the amount of polymer determined for homolysates using ultracentrifuge sedimentation. In general, these results on intracellular gelation are in good agreement with results predicted by cell-free studies extrapolated to the high intracellular hemoglobin concentration (34 g/dl). The significance of the detection of polymer at high oxygen saturation of SS erythrocytes is discussed in terms of morphologic deformation and erythrocyte transit through the circulation. We propose that intracellular polymerization is important in abnormal cell rheology and manifestation of disease.

摘要

利用碳-13核磁共振技术,我们研究了血红蛋白S的聚合作用以及SS型和AS型红细胞中的细胞内聚合现象。对于SS型红细胞,在高氧饱和度(大于90%)时可检测到聚合物,并且随着氧饱和度降低至完全脱氧时总血红蛋白的70%,聚合物含量增加。这些结果与血红蛋白S在细胞内34 g/dl的高浓度下的非理想行为一致。还测定了AS型红细胞中聚合物的含量与氧饱和度的函数关系,其结果与使用超速离心沉降法测定的同型裂解物中的聚合物含量相符。总体而言,这些关于细胞内凝胶化的结果与通过无细胞研究外推至细胞内高血红蛋白浓度(34 g/dl)所预测的结果高度一致。从形态变形和红细胞在循环中的转运角度讨论了在SS型红细胞高氧饱和度下检测到聚合物的意义。我们认为细胞内聚合作用在异常细胞流变学和疾病表现中具有重要作用。

相似文献

1
Determination of sickle hemoglobin polymer in SS and AS erythrocytes.SS型和AS型红细胞中镰状血红蛋白聚合物的测定。
Blood Cells. 1982;8(2):225-35.
2
Intracellular polymerization of sickle hemoglobin. Effects of cell heterogeneity.镰状血红蛋白的细胞内聚合。细胞异质性的影响。
J Clin Invest. 1983 Sep;72(3):846-52. doi: 10.1172/JCI111055.
3
Intracellular polymerization of sickle hemoglobin: disease severity and therapeutic goals.镰状血红蛋白的细胞内聚合:疾病严重程度与治疗目标
Prog Clin Biol Res. 1987;240:381-91.
4
Non-uniformity of intracellular polymer formation in sickle erythrocytes: possible correlation with severity of hemolytic anemia.镰状红细胞内聚合物形成的不均匀性:与溶血性贫血严重程度的可能关联。
Am J Pediatr Hematol Oncol. 1984 Spring;6(1):46-50.
5
Determination of deoxyhemoglobin S polymer in sickle erythrocytes upon deoxygenation.脱氧时镰状红细胞中脱氧血红蛋白S聚合物的测定。
Proc Natl Acad Sci U S A. 1980 Sep;77(9):5487-91. doi: 10.1073/pnas.77.9.5487.
6
Electron microscopic studies of the intracellular polymerization of sickle hemoglobin.镰状血红蛋白细胞内聚合的电子显微镜研究。
Blood Cells. 1988;13(3):359-76.
7
Polymerization of sickle cell hemoglobin at arterial oxygen saturation impairs erythrocyte deformability.在动脉血氧饱和度下镰状细胞血红蛋白的聚合会损害红细胞的可变形性。
J Clin Invest. 1988 Jun;81(6):1669-74. doi: 10.1172/JCI113504.
8
Phosphorus-31 nuclear magnetic resonance studies of human red blood cells.人体红细胞的磷-31核磁共振研究。
Blood Cells. 1982;8(2):245-61.
9
Effects of carbon dioxide and pH variations in vitro on blood respiratory functions, red blood cell volume, transmembrane pH gradients, and sickling in sickle cell anemia.体外二氧化碳和pH值变化对镰状细胞贫血患者血液呼吸功能、红细胞体积、跨膜pH梯度及镰变的影响。
J Lab Clin Med. 1984 Aug;104(2):146-59.
10
Viscoelastic properties of sickle cells and hemoglobin.镰状细胞和血红蛋白的粘弹性特性。
Blood Cells. 1982;8(1):53-64.

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