Zakharov S F, Shandala A M, Shcheglova M V, Gromov P S, Insarova N G, Sychova V A, Markova E D, Shishkin S S, Ivanova-Smolenskaia I A
Vopr Med Khim. 1990 Nov-Dec;36(6):71-3.
Erythrocytes of healthy volunteers and of patients with hereditary chorea were studied. Evaluation of the state of cellular membrane was carried out by measuring osmotic resistance, activity of Na+, K(+)-ATPase and protein composition. In the patients osmotic resistance of erythrocytes was distinctly decreased down to 66.3 +/- 3.3, while the Na+, K(+)-ATPase activity was increased 4-fold as compared with controls. Protein composition of erythrocyte membranes, studied by means of two-dimensional electrophoresis, was similar both in healthy persons and in patients with hereditary chorea when the electrophoretograms were analyzed visually. An additional protein with Mr = 30,000 and r-1-0.25 was detected in one of the patients. Slowly sedimenting fraction of erythrocytes was found in almost all the patients with hereditary chorea when erythrocytes aging was studied by means of fractionation in Ficoll density gradient. The fraction was not observed in healthy persons. These data suggest that the cell membranes in Huntington's chorea are altered as compared with normal state.
对健康志愿者和遗传性舞蹈病患者的红细胞进行了研究。通过测量渗透压抵抗力、Na +、K(+)-ATP酶活性和蛋白质组成来评估细胞膜的状态。患者红细胞的渗透压抵抗力明显降低至66.3±3.3,而Na +、K(+)-ATP酶活性与对照组相比增加了4倍。通过二维电泳研究红细胞膜的蛋白质组成,当直观分析电泳图时,健康人和遗传性舞蹈病患者的情况相似。在其中一名患者中检测到一种额外的蛋白质,其Mr = 30,000且r-1-0.25。当通过在Ficoll密度梯度中分级分离来研究红细胞老化时,几乎所有遗传性舞蹈病患者都发现了红细胞的缓慢沉降部分。在健康人中未观察到该部分。这些数据表明,与正常状态相比,亨廷顿舞蹈病患者的细胞膜发生了改变。