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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。

Cystic fibrosis: normal volumes of red cells. Model experiments on the pathomechanism of the disease.

作者信息

Emrich H M, Heitz J, Katz S, Thom R

出版信息

Eur J Pediatr. 1976 Jul 12;122(4):293-6. doi: 10.1007/BF00481510.

DOI:10.1007/BF00481510
PMID:59666
Abstract

Normal values of mean cell volume (M.C.V.) and of distribution of single cell volumes (S.C.V.) have been observed in erythrocytes of patients with cystic fibrosis using an electronical particle-volume analyzer (sheath flow detector). From these results a strong defect in red cell salt transport seems improbable. Valinomucin induces shrinking (by increase of K+ permeability) and gramicidin D induces swelling (by increase of Na+ permeability) of normal erythrocytes. Addition of C.F. sweat to normal erythrocytes induces no volume change. From this result no influence of the "C.F. factor" on passive ion permeability is concluded.

摘要

使用电子颗粒体积分析仪(鞘流检测器)观察了囊性纤维化患者红细胞的平均细胞体积(M.C.V.)和单细胞体积分布(S.C.V.)的正常值。从这些结果来看,红细胞盐转运存在严重缺陷的可能性不大。缬氨霉素可诱导正常红细胞收缩(通过增加钾离子通透性),短杆菌肽D可诱导正常红细胞肿胀(通过增加钠离子通透性)。向正常红细胞中添加囊性纤维化患者的汗液不会引起体积变化。由此结果得出“囊性纤维化因子”对被动离子通透性没有影响的结论。

相似文献

1
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.
2
The influence of gramicidin A and valinomycin of the permeability of mammalian erythrocytes.短杆菌肽A和缬氨霉素对哺乳动物红细胞通透性的影响。
Acta Biochim Pol. 1975;22(4):269-78.
3
Chloride transport by self-exchange and by KCl salt diffusion in gramicidin-treated red blood cells.短杆菌肽处理的红细胞中通过自身交换和氯化钾盐扩散进行的氯离子转运。
Acta Physiol Scand. 1979 Nov;107(3):193-203. doi: 10.1111/j.1748-1716.1979.tb06463.x.
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Cl- permeabilities in red blood cells and peripheral blood lymphocytes from cystic fibrosis and control subjects.囊性纤维化患者及对照受试者红细胞和外周血淋巴细胞中的氯离子通透性。
Pediatr Res. 1984 Dec;18(12):1336-9. doi: 10.1203/00006450-198412000-00024.
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Voltage dependence of DIDS-insensitive chloride conductance in human red blood cells treated with valinomycin or gramicidin.用缬氨霉素或短杆菌肽处理的人红细胞中对DIDS不敏感的氯电导的电压依赖性
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Electrodiffusion, barrier, and gating analysis of DIDS-insensitive chloride conductance in human red blood cells treated with valinomycin or gramicidin.缬氨霉素或短杆菌肽处理的人红细胞中对4,4'-二异硫氰基二苯乙烯-2,2'-二磺酸(DIDS)不敏感的氯电导的电扩散、屏障及门控分析
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Pediatr Res. 1988 Oct;24(4):495-8. doi: 10.1203/00006450-198810000-00016.
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Rapid electrogenic sulfate-chloride exchange mediated by chemically modified band 3 in human erythrocytes.人红细胞中化学修饰的带3介导的快速电生硫酸根-氯离子交换
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Determination of membrane potentials in human and Amphiuma red blood cells by means of fluorescent probe.利用荧光探针测定人和蚓螈红细胞的膜电位。
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10
23Na and 39K NMR studies of ion transport in human erythrocytes.人体红细胞中离子转运的23Na和39K核磁共振研究。
Proc Natl Acad Sci U S A. 1985 Feb;82(4):1099-103. doi: 10.1073/pnas.82.4.1099.

本文引用的文献

1
Abnormal electrolyte composition of sweat in cystic fibrosis of the pancreas; clinical significance and relationship to the disease.胰腺囊性纤维化患者汗液中电解质成分异常;临床意义及其与疾病的关系。
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Sodium transport: inhibitory factor in sweat of patients with cystic fibrosis.
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Sweat composition in relation to rate of sweating in patients with cystic fibrosis of the pancreas.胰腺囊性纤维化患者汗液成分与出汗速率的关系。
Pediatr Res. 1968 Nov;2(6):464-78. doi: 10.1203/00006450-196811000-00004.
4
Further evidcence for an optical response of chloroplast bulk pigments to a light induced electrical field in photosynthesis.叶绿体大量色素对光合作用中光诱导电场产生光学响应的进一步证据。
Z Naturforsch B. 1969 Sep;24(9):1144-6. doi: 10.1515/znb-1969-0912.
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Erythrocyte transport function in cystic fibrosis.
Pediatr Res. 1974 May;8(5):594-7. doi: 10.1203/00006450-197405000-00008.
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Changes in erythrocyte membrane ATPase in patients with cystic fibrosis of the pancreas.胰腺囊性纤维化患者红细胞膜ATP酶的变化
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Depressed (Ca++)-transport ATPase in cystic fibrosis erythrocytes.囊性纤维化红细胞中(钙离子)转运ATP酶活性降低。
Biochem Biophys Res Commun. 1970 Aug 11;40(3):505-9. doi: 10.1016/0006-291x(70)90930-7.
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Red-cell transport defect in patients with cystic fibrosis and in their parents.
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Antibiotic-mediated transport of alkali ions across lipid barriers.抗生素介导的碱金属离子跨脂质屏障运输。
Proc Natl Acad Sci U S A. 1967 Nov;58(5):1949-56. doi: 10.1073/pnas.58.5.1949.