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Red-cell transport defect in patients with cystic fibrosis and in their parents.

作者信息

Balfe J W, Cole C, Welt L G

出版信息

Science. 1968 Nov 8;162(3854):689-90. doi: 10.1126/science.162.3854.689.

DOI:10.1126/science.162.3854.689
PMID:4235015
Abstract

The ouabain-sensitive and the ethacrynic acid-sensitive sodium efflux from erythrocytes of patients with cystic fibrosis are both decreased. Furthermore, the ouabain-sensitive adenosine triphosphatase activity is diminished in the red blood cell ghosts of these patients. Perhaps of greater significance is the fact that ethacrynic acid-sensitive sodium efflux is clearly diminished in the erythrocytes of the asymptomatic parents of these sick children. This defect in sodium transport may be valuable for detecting the heterozygous carrier state.

摘要

相似文献

1
Red-cell transport defect in patients with cystic fibrosis and in their parents.
Science. 1968 Nov 8;162(3854):689-90. doi: 10.1126/science.162.3854.689.
2
Letter: Na+ transport defect in cystic fibrosis.信件:囊性纤维化中的钠离子转运缺陷。
Nature. 1974 May 3;249(452):94-5. doi: 10.1038/249094c0.
3
The relationship of membrane ATPase activity to ouabain-insensitive sodium transport in human red cells.
Can J Physiol Pharmacol. 1973 Sep;51(9):642-8. doi: 10.1139/y73-097.
4
Ion movements in human red cells independent of the sodium pump.人类红细胞中与钠泵无关的离子运动。
J Physiol. 1969 May;202(1):111-31. doi: 10.1113/jphysiol.1969.sp008798.
5
Drugs affecting sodium transport in human erythrocyte ghosts.影响人红细胞血影中钠转运的药物。
J Pharmacol Exp Ther. 1970 Apr;172(2):211-23.
6
Effect of saliva from cystic fibrosis patients and from normal subjects on red blood cell sodium transport.
Pediatr Res. 1978 Jan;12(1):1-3. doi: 10.1203/00006450-197801000-00001.
7
Intracellular sodium, potassium and magnesium concentration, ouabain-sensitive 86rubidium-uptake and sodium-efflux and Na+, K+-cotransport activity in erythrocytes of normal male subjects studied on two occasions.对正常男性受试者的红细胞进行了两次研究,测定了细胞内钠、钾和镁的浓度、哇巴因敏感的86铷摄取、钠外流以及钠钾协同转运活性。
Methods Find Exp Clin Pharmacol. 1986 Sep;8(9):525-33.
8
Inheritance of an increased sodium pump in human red cells.人类红细胞中钠泵增加的遗传现象。
Nature. 1969 Mar 29;221(5187):1222-4. doi: 10.1038/2211222a0.
9
Erythrocyte transport function in cystic fibrosis.
Pediatr Res. 1974 May;8(5):594-7. doi: 10.1203/00006450-197405000-00008.
10
Ouabain-uninhibited sodium transport in human erythrocytes. Evidence against a second pump.哇巴因对人红细胞钠转运的非抑制作用。反对存在第二个泵的证据。
J Clin Invest. 1973 Mar;52(3):658-70. doi: 10.1172/JCI107227.

引用本文的文献

1
Na and K dependence of the Na/K pump in cystic fibrosis fibroblasts.囊性纤维化成纤维细胞中钠钾泵对钠和钾的依赖性
Proc Natl Acad Sci U S A. 1981 Nov;78(11):7143-6. doi: 10.1073/pnas.78.11.7143.
2
Cystic fibrosis of the pancreas--new clues to the metabolic riddle.胰腺囊性纤维化——代谢之谜的新线索
Calif Med. 1969 May;110(5):432-4.
3
The effects of transport inhibitors on sodium outflux and influx in red blood cells: evidence for exchange diffusion.转运抑制剂对红细胞钠外流和内流的影响:交换扩散的证据。
J Clin Invest. 1970 Oct;49(10):1804-14. doi: 10.1172/JCI106398.
4
The effects of an inhibitor of Na plus -K plus -active transport on the secretory activity of rat submaxillary gland cell cultures.钠钾主动转运抑制剂对大鼠下颌下腺细胞培养物分泌活性的影响。
Z Zellforsch Mikrosk Anat. 1971;117(3):314-21. doi: 10.1007/BF00324807.
5
Cystic fibrosis of the pancreas. A study in cell culture.胰腺囊性纤维化。细胞培养研究。
J Exp Med. 1969 Apr 1;129(4):775-93. doi: 10.1084/jem.129.4.775.
6
Studies on the synthesis of plasma membrane proteins of fibroblasts from patients with cystic fibrosis.囊性纤维化患者成纤维细胞质膜蛋白合成的研究。
Am J Pathol. 1975 Aug;80(2):317-28.
7
Cystic fibrosis: normal volumes of red cells. Model experiments on the pathomechanism of the disease.囊性纤维化:红细胞体积正常。关于该疾病发病机制的模型实验。
Eur J Pediatr. 1976 Jul 12;122(4):293-6. doi: 10.1007/BF00481510.