光诱导红细胞生成性原卟啉症患者红细胞中原卟啉的释放。
Light-induced protoporphyrin release from erythrocytes in erythropoietic protoporphyria.
作者信息
Sandberg S, Brun A
出版信息
J Clin Invest. 1982 Sep;70(3):693-8. doi: 10.1172/jci110664.
The photohemolysis of normal erythrocytes incubated with protoporphyrin is reduced in the presence of albumin. When globin is added to normal erythrocytes loaded with protoporphyrin, protoporphyrin is bound to globin. During irradiation protoporphyrin moves from globin to the erythrocyte membrane and photohemolysis is initiated. Erythrocytes in patients with erythropoietic protoporphyria contain large amounts of protoporphyrin bound to hemoglobin. Upon irradiation of these cells in the absence of albumin, 40% of protoporphyrin and 80% of hemoglobin is released after 240 kJ/m2. The released protoporphyrin is hemoglobin bound. In contrast, when albumin is present only 8% of hemoglobin is released whereas protoporphyrin is released to 76%. The released protoporphyrin is albumin bound. A hypothesis for the release of erythrocyte protoporphyrin in erythropoietic protoporphyria without simultaneous hemolysis is proposed. Upon irradiation protoporphyrin photodamages its binding sites on hemoglobin, moves through the plasma membrane, and is bound to albumin in plasma.
在白蛋白存在的情况下，与原卟啉一起孵育的正常红细胞的光溶血作用会降低。当向负载有原卟啉的正常红细胞中添加珠蛋白时，原卟啉会与珠蛋白结合。在照射过程中，原卟啉从珠蛋白转移到红细胞膜上并引发光溶血。红细胞生成性原卟啉症患者的红细胞含有大量与血红蛋白结合的原卟啉。在无白蛋白的情况下照射这些细胞，在240 kJ/m²后，40%的原卟啉和80%的血红蛋白会被释放出来。释放出的原卟啉与血红蛋白结合。相比之下，当存在白蛋白时，只有8%的血红蛋白被释放，而原卟啉的释放量为76%。释放出的原卟啉与白蛋白结合。提出了一个关于红细胞生成性原卟啉症中红细胞原卟啉释放而无同时溶血的假说。在照射时，原卟啉对其在血红蛋白上的结合位点造成光损伤，穿过质膜，并与血浆中的白蛋白结合。
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