CAJ | 학술논문

目的为探讨海南黎族β一地中海贫血红细胞膜蛋白是否存在异常，我们观察了33例生活在海南保亭县的β一地中海贫血病人。方法用十二烷基磺酸钠一聚丙烯酰胺凝胶电泳技术分离蛋白；激光密度扫描量化各蛋含量。结果β一地中海贫血与正常对照比较发现血影蛋白(spectrin)，4.5蛋白和球蛋白量明显增加(P＜0.05)。结论β一地中海贫血病人红细胞膜蛋白成份以及它们与血蛋白的结合存在异常。这些改变可能使得红细胞僵硬、变形性降低而导致溶血。
목적위탐토해남려족β일지중해빈혈홍세포막단백시부존재이상，아문관찰료33례생활재해남보정현적β일지중해빈혈병인。방법용십이완기광산납일취병희선알응효전영기술분리단백；격광밀도소묘양화각단함량。결과β일지중해빈혈여정상대조비교발현혈영단백(spectrin)，4.5단백화구단백량명현증가(P＜0.05)。결론β일지중해빈혈병인홍세포막단백성빈이급타문여혈단백적결합존재이상。저사개변가능사득홍세포강경、변형성강저이도치용혈。
Objective To determine the composition of abnormal red cell membrane skeletin. Methods By sodium dodecyl sulphate polyacrylamide gel electrophoresis of ghostcorpuscles, we quantified the amount of protein by densitometric evaluation.Results The results showed that in β-thalassernia, the amount of spectrin, 4.5 protein and globin significantly increased compared with the controls (26.05 + 1.46, 21.69 + 1.86; 22.87 + 5.61, 12.99 +2.33; 15.23+3.31 and 4.97+2.73, respectively, P＜0.05).Conclusion These data suggest that the erythrocyte membrane protein composition matched with globin in patients with β-thalassemia of the racial Li minority are different from the normal control. These factors increase rigidity but decrease deformability of the β-thalassemic red cell membrane, which may lead to hemolytic anemia.