Missing band 7 membrane protein in two patients with high Na, low K erythrocytes.
Missing band 7 membrane protein in two patients with high Na, low K erythrocytes.
复制标题
两名高 Na、低 K 红细胞患者的带 7 膜蛋白缺失。
DOI：
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发表时间：
1982
影响因子：
15.9
通讯作者：
W. Mentzer
中科院分区：
文献类型：
--
作者：
W. Lande;P. Thiemann;W. Mentzer
我们调查了两名先天性溶血性贫血患者的红细胞膜蛋白，由于增加了红细胞膜的渗透性Na和K（遗传性口细胞增多症和cryohydrocytosis）。一维十二烷基硫酸钠（SDS）凝胶电泳将条带7红细胞膜蛋白分解为三种组分，分子量约为30，000、28，000和26，000。28，000-道尔顿组分在两名渗透性疾病患者中均降低。二维电泳（第一维的非平衡pH梯度电泳结合第一维的SDS凝胶电泳结合第二维的SDS凝胶电泳）将正常红细胞膜中的28，000-道尔顿组分分解为两种具有不同等电点的蛋白质，命名为22 x 8和60 x 8。在患有遗传性口细胞增多症和冷冻水胞症的患者中，22 × 8完全不存在，而60 × 8则照常检测到。相反，所有的带7蛋白（包括22 × 8）总是存在于正常受试者和网织红细胞增多症对照的调查。在患有遗传性气孔增多症和冷冻水胞症的患者中缺失带7蛋白的独特发现提出了这种蛋白的缺失是导致这些疾病中Na和K渗透性增加的可能性。
We investigated the erythrocyte membrane proteins of two patients with congenital hemolytic anemia due to increased permeability of the erythrocyte membrane to Na and K (hereditary stomatocytosis and cryohydrocytosis). One-dimensional sodium dodecyl sulfate (SDS) gel electrophoresis resolved the band 7 erythrocyte membrane proteins into three components with approximate molecular weights of 30,000, 28,000, and 26,000. The 28,000-dalton component was decreased in both patients with permeability disorders. Two-dimensional electrophoresis (nonequilibrium pH gradient electrophoresis in the first dimension combined with SDS gel electrophoresis in the first dimension combined with SDS gel electrophoresis in the second dimension) resolved the 28,000-dalton component from normal erythrocyte membranes into two proteins with different isoelectric points, designated 22 x 8 and 60 x 8. In the patients with hereditary stomatocytosis and cryohydrocytosis, 22 x 8 was completely absent, whereas 60 x 8 was detected as usual. In contrast, all the band 7 proteins (including 22 x 8) were invariably present in a survey of normal subjects and reticulocytosis controls. The unique finding of a missing band 7 protein in the patients with hereditary stomatocytosis and cryohydrocytosis raises the possibility that the absence of this protein is responsible for the increased Na and K permeability in these disorders.
DOI：
10.1016/0005-2795(81)90111-2
发表时间：
1981-10
期刊：
Biochimica et biophysica acta
影响因子：
--
作者：
H. Malech;V. Marchesi
通讯作者：
H. Malech;V. Marchesi
影响因子：
158.5
作者：
AGRE, P;ORRINGER, EP;BENNETT, V
通讯作者：
BENNETT, V