Abnormal permeability pathways in human red blood cells
Abnormal permeability pathways in human red blood cells
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DOI：
10.1016/j.bcmd.2007.02.011
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发表时间：
2007-07-01
影响因子：
2.3
通讯作者：
Gibson, J. S.
中科院分区：
文献类型：
--
作者：
Ellory, J. C.;Robinson, H. C.;Gibson, J. S.
已对导致人类红细胞(RBC)异常通透性途径的许多情况进行了调查。在镰状细胞病(SCD)中，红细胞含有HBs，而不是正常的HBA。当去氧时，一条称为P-镰刀的异常电导通路被激活，这主要是通过允许钙离子进入并随后激活Gardos通道，导致细胞脱水。镰刀形红细胞的全细胞膜片钳记录显示，正常红细胞不存在脱氧诱导的电导，这与P-镰刀形红细胞的一些特性相同：相当于Na+和K+通透性，显著的钙电导，DIDS和锌离子的部分抑制。Gd3+对正常和镰状红细胞的电导均有明显的抑制作用。此外，除氧的镰状细胞，而不是含氧的细胞或正常的红细胞，无论氧分如何，都会在等渗的非电解质溶液中发生溶血。非电解质进入被放射性同位素证实，而溶血被DIDS抑制。这些发现表明，在某些情况下，P-镰刀也可能对非电解质具有渗透性。最后，来自某些遗传性口腔细胞增多症患者的红细胞有一个突变的带3，它似乎能够作为一价阳离子的传导途径。这些结果加深了我们对红细胞异常通透途径的理解。(C)2007 Elsevier Inc.保留所有权利。
A number of situations that result in abnormal permeability pathways in human red blood cells (RBCs) have been investigated. In sickle cell disease (SCD), RBCs contain HbS, rather than the normal HbA. When deoxygenated, an abnormal conductance pathway, termed P-sickle, is activated, which contributes to cell dehydration, largely through allowing Ca2+ entry and subsequent activation of the Gardos channel. Whole-cell patch-clamp recordings from sickle RBCs show a deoxygenated-induced conductance, absent from normal RBCs, which shares some of the properties of P-sickle: equivalent Na+ and K+ permeability, significant Ca2+ conductance, partial inhibition by DIDS and also Zn2+. Gd3+ markedly attenuates conductance in both normal and sickle RBCs. In addition, deoxygenated sickle cells, but not oxygenated ones or normal RBCs regardless of the oxygen tension, undergo haemolysis in isosmotic non-electrolyte solutions. Non-electrolyte entry was confirmed radioisotopically whilst haemolysis was inhibited by DIDS. These findings suggest that under certain circumstances P-sickle may also be permeable to non-electrolytes. Finally, RBCs from certain patients with hereditary stomatocytosis have a mutated band 3, which appears able to act as a conductance pathway for univalent cations. These results extend our understanding of the abnormal permeability pathways of RBCs. (c) 2007 Elsevier Inc. All rights reserved.