Studies on abnormal hemoglobins. VIII. The gelling phenomenon of sickle cell hemoglobin: its biologic and diagnostic significance.
Studies on abnormal hemoglobins. VIII. The gelling phenomenon of sickle cell hemoglobin: its biologic and diagnostic significance.
复制标题
异常血红蛋白的研究。
DOI：
10.1182/blood.v8.11.1008.1008
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发表时间：
1953
期刊：
影响因子：
20.3
通讯作者：
L. Singer
中科院分区：
文献类型：
--
作者：
Karl Singer;L. Singer
1.当含有足够浓缩的镰状细胞血红蛋白的溶液暴露在持续流动的二氧化碳气体中时，溶血物形成凝胶。这种凝胶现象表明S血红蛋白的存在，在没有S色素的情况下，任何其他类型的人血红蛋白都不能获得这种凝胶现象。S的最低血红蛋白浓度(GM。仍能引发凝胶现象的溶血物的最低凝固点被指定为1%。2.研制了一种在标准条件下分析胶凝现象的简易装置。结果表明，镰状细胞性状的红细胞(含A+S血红蛋白)、“C变异型”红细胞(含C+S血红蛋白)和镰状细胞贫血细胞(含S+F血红蛋白)制备的溶血物的最低凝集点有明显差异。进一步的实验表明，A型血红蛋白的存在降低了凝胶形成所需的S色素的最低含量，C型血红蛋白甚至进一步降低了这一数量。F-血红蛋白似乎对凝胶现象没有显著影响。血清白蛋白还能降低S凝集所需的血红蛋白含量。3.镰刀状红细胞被视作S血红蛋白胶状或凝胶，受与S化合物相互作用的伴生色素的特殊影响。因此，在镰状细胞性状中，镰状试验阳性不仅是由S血红蛋白的存在引起的，也是由于它与A血红蛋白的相互作用引起的。只有在镰状细胞贫血细胞中，镰状细胞似乎完全依赖于S血红蛋白分子的相互作用。4.从不同类型的镰状红细胞制备的溶血物的最低凝固点的明显差异构成了诊断凝胶试验的基础，该试验明显区分镰状细胞性贫血和镰状细胞特性红细胞。通过这个程序，也可以检测到镰状细胞病的非典型病例，例如，那些红细胞含有C血红蛋白的病例。
1. When sufficiently concentrated sickle cell hemoglobin containing solutions are exposed to a constant stream of CO2 gas, the hemolysates gel. This gelling phenomenon is indicative of the presence of S hemoglobin and cannot be obtained with any other type of human hemoglobin in the absence of S pigment. The lowest S hemoglobin concentration (Gm. per cent) of a hemolysate at which the gelling phenomenon can still be elicited is designated as its lowest gelling point. 2. A simple apparatus was developed to analyze the gelling phenomenon under standardized conditions. It could be shown that the lowest gelling points of hemolysates prepared from erythrocytes of the sickle cell trait (containing A + S hemoglobins), of the "C variant" (containing C + S hemoglobins), and from sickle cell anemia cells (containing S + F hemoglobins) differ distinctly. Further experiments suggest that the presence of A hemoglobin decreases the minimal amount of S pigment required for gel formation, and that type C hemoglobin reduces this amount even further. F hemoglobin seems to exert no significant influence on the gelling phenomenon. Serum albumin is also capable of decreasing the amount of S hemoglobin required for gelation. 3. A sickled erythrocyte is visualized as an S hemoglobin tactoid or gel, specifically influenced by the companion pigment which interacts with the S compound. Thus, in the sickle cell trait, a positive sickling test is not only caused by the presence of S hemoglobin, but also by its interaction with A hemoglobin. Only in the sickle cell anemia cells does sickling seem to depend solely upon the interaction of the S hemoglobin molecules. 4. The readily demonstrable differences of the lowest gelling points of hemolysates prepared from the various types of sickling red cells form the basis of the diagnostic gelling test which distinguishes sharply between sickle cell anemia and sickle cell trait erythrocytes. By this procedure atypical cases of sickle cell disease, for example, those whose erythrocytes contain C hemoglobin, may also be detected.