Increased erythrocyte cation permeability in thalassemia and conditions of marrow stress.
Increased erythrocyte cation permeability in thalassemia and conditions of marrow stress.
复制标题
地中海贫血和骨髓应激状态下红细胞阳离子通透性增加。
DOI：
10.1172/jci110140
复制
发表时间：
1981
期刊：
影响因子：
--
通讯作者：
J. Wiley
中科院分区：
文献类型：
--
作者：
J. Wiley
对未输血的α-或β-地中海贫血患者的红细胞的钙、钠通透性进行了研究，并与网织红细胞增多症的成熟红细胞或对照细胞进行了比较。同位素钠(+)流入增加，平均比正常增加四倍，比富网织红细胞对照组增加三倍。与哇巴因孵育的地中海贫血细胞的Na(+)被动净渗漏也随着(22)Na(+)内流的增加而增加。红细胞Na(+)、K(+)浓度及单位体积细胞含水量正常。[(3)H]哇巴因结合技术测定细胞膜上活性阳离子泵的数量比正常增加了2.6到9.9倍。本文研究了细胞内钙离子的内向运动，细胞内的钙离子泵是由耗竭ATP或镁离子引起的。ATP耗竭的地中海贫血细胞的钙摄取比正常增加12倍，比富网织红细胞的对照增加3.6倍。缺镁地中海贫血细胞对Ca(2+)的摄取也高于正常，证实本病患者红细胞Ca(2+)通透性增高。渗透脆性测定表明，地中海贫血红细胞的表面积与体积比比成熟红细胞增加了15%~25%。地中海贫血红细胞被动阳离子通透性的增加既不能用网织红细胞增多也不能用这些细胞表面积与体积比的增加来解释。此外，先天性红细胞生成性贫血(CDA 2型)患者的红细胞Na(+)和Ca(2+)内流分别是正常的2倍和14倍。地中海贫血和先天性红细胞生成障碍性贫血红细胞阳离子通量和阳离子泵数目的增加与膜不成熟的假说一致，该假说是由快速的骨髓转运时间引起的，这一概念以前被提出来解释I抗原在这些细胞上的持久性。
Calcium and sodium permeability of erythrocytes from patients with untransfused alpha- or beta- thalassemia major has been studied and compared to mature erythrocytes or control cells with comparable reticulocytosis. Isotopic Na(+) influx was increased a mean fourfold greater than normals and threefold greater than reticulocyte rich control. Passive net leak of Na(+) into thalassemic cells incubated with ouabain was also increased corresponding to their greater (22)Na(+) influx. Erythrocyte Na(+) and K(+) concentrations and cell water content per unit volume of cells were normal. Quantitation of active cation pumps in the cell membrane by the technique of [(3)H]ouabain binding showed a 2.6- to 9.9-fold increase above normal. Inward Ca(2+) movement was studied in cells with absent Ca(2+) pumping produced by depletion of either ATP or Mg(2+)-ions. Calcium uptake by ATP depleted thalassemic cells was increased 12-fold above normals and 3.6-fold above reticulocyte-rich controls. The Ca(2+) uptake by Mg(2+)-depleted thalassemic cells was also increased above normal confirming that erythrocyte Ca(2+) permeability is increased in this disease. Osmotic fragility measurements show that the surface area to volume ratio of thalassemic erythrocytes was increased by 15 to 25% above mature erythrocytes. The increased passive cation permeability of thalassemic erythrocytes cannot be explained by either reticulocytosis or an increased surface area to volume ratio of these cells. Moreover, erythrocyte Na(+) and Ca(2+) influxes in congenital dyserythropoietic anemia (CDA type 2) were increased 2- and 14-fold, respectively, above normal. The increased cation fluxes and cation pump numbers in thalassemic and congenital dyserythropoietic anemia erythrocytes are consistent with the hypothesis of membrane immaturity arising from rapid marrow transit times, a concept previously advanced to explain the persistence of i-antigen on these cells.