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磷脂酶处理前后人类肌肉萎缩症患者红细胞的渗透稳定性

Osmotic stability of erythrocytes in human muscular dystrophy before and after phospholipase treatment.

作者信息

Ruitenbeek W, Edixhoven M J, Scholte H R

出版信息

Clin Chim Acta. 1979 Jun 15;94(3):259-66. doi: 10.1016/0009-8981(79)90073-1.

Abstract

The stability of washed erythrocytes from patients with muscular dystrophy was determined in hypotonic phosphate buffered sodium chloride. Control cells were more stable than cells from Duchenne and myotonic patients. After pretreatment of the cells with phospholipase from pancreas, snake venom or bee venom in the presence of 14 mmol/l Ca2+, the order of osmotic stability in the 3 groups was not changed. In isotonic phosphate buffered NaCl, however, the erythrocytes of the myotonic patients were much more stable than the cells of the Duchenne and the control group. The lytic process was further studied in control cells with pancreatic phospholipase. 21 +/- 3 (S.E.M.) % of the cells were lysed. This process was (partly) prevented by omitting the phospholipase, by replacement of Na+ by K+ or Li+, by lowering the Ca2+ concentration, by omitting phosphate, by ouabain, by glucose, by ribose, by sucrose, by tetrodotoxin, a Na+-transport inhibitor. Blocking of the Ca2+ transport by La3+ or mersalyl, greatly stimulated the lytic process.

摘要

在低渗磷酸盐缓冲氯化钠溶液中测定了肌营养不良症患者洗涤红细胞的稳定性。对照细胞比杜兴氏和强直性肌营养不良患者的细胞更稳定。在14 mmol/l Ca2+存在的情况下,用胰腺磷脂酶、蛇毒或蜂毒对细胞进行预处理后,三组的渗透稳定性顺序未改变。然而,在等渗磷酸盐缓冲氯化钠溶液中,强直性肌营养不良患者的红细胞比杜兴氏患者和对照组的细胞稳定得多。用胰腺磷脂酶对对照细胞的溶血过程进行了进一步研究。21±3(标准误)%的细胞发生溶血。省略磷脂酶、用K+或Li+替代Na+、降低Ca2+浓度、省略磷酸盐、使用哇巴因、葡萄糖、核糖、蔗糖、河豚毒素(一种Na+转运抑制剂)可(部分)阻止这一过程。La3+或汞撒利阻断Ca2+转运可极大地刺激溶血过程。

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Alteration in erythrocyte membrane structure in Duchenne muscular dystrophy.
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