REGULATION OF ERYTHROCYTE CATION AND WATER-CONTENT IN SICKLE-CELL-ANEMIA
REGULATION OF ERYTHROCYTE CATION AND WATER-CONTENT IN SICKLE-CELL-ANEMIA
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DOI：
10.1126/science.3961486
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发表时间：
1986-04-18
期刊：
影响因子：
56.9
通讯作者：
TOSTESON, DC
中科院分区：
文献类型：
--
作者：
BRUGNARA, C;BUNN, HF;TOSTESON, DC
镰状细胞病中的病理生理学事件严重依赖于血红蛋白S的细胞内浓度，其与细胞阳离子和水含量成反比。SS纯合子暴露于氧气或一氧化碳的红细胞减少其钾和水含量通过钾运输的途径，这是由细胞肿胀和减少内部pH值激活。这条途径是不抑制哇巴因无论有或没有布美他尼。当SS erthyroidcells分离，根据密度，pH值和体积依赖性钾运输是最大的密度最小的部分，并减少在denatrium细胞。这一途径不依赖于镰状血红蛋白的聚合，可能在调节SS红细胞的阳离子和水含量方面很重要。
The pathophysiological events in sickle cell disease are critically dependent on the intracellular concentration of hemoglobin S, which varies inversely with cell cation and water content. Erythrocytes of SS homozygotes exposed to oxygen or carbon monoxide decrease their potassium and water content through a pathway for potassium transport that is activated by both cell swelling and decrease in internal pH. This pathway is not inhibited by ouabain either with or without bumetanide. When SS erthyrocytes were separated according to density, the pH- and volume-dependent potassium transport was greatest in the least dense fraction and was reduced in the densest cells. This pathway, which does not depend on polymerization of sickle hemoglobin, may be important in regulating the cation and water content of SS erythrocytes.