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来自杜兴氏肌营养不良症患者的培养肌肉细胞在静息时以及胆碱能刺激后的游离细胞质钙离子浓度增加。

Free cytoplasmic Ca++ at rest and after cholinergic stimulus is increased in cultured muscle cells from Duchenne muscular dystrophy patients.

作者信息

Mongini T, Ghigo D, Doriguzzi C, Bussolino F, Pescarmona G, Pollo B, Schiffer D, Bosia A

机构信息

Clinica Neurologica II, Università degli Studi, Torino, Italy.

出版信息

Neurology. 1988 Mar;38(3):476-80. doi: 10.1212/wnl.38.3.476.

Abstract

We used a fluorescent dye, quin 2, to measure intracellular free calcium ([Ca++]i) in cultured skeletal muscle cells and skin fibroblasts from five Duchenne muscular dystrophy (DMD) patients and from five controls. We observed an enhanced [Ca++]i level, at rest and after acetylcholine (ACh) stimulation, in DMD muscle cells, but we did not detect any difference between DMD and normal skin fibroblasts. The abnormally higher [Ca++]i transient induced by ACh suggests that it plays a critical role in muscle degeneration. The skin fibroblast results suggests that there is no generalized membrane defect.

摘要

我们使用荧光染料喹啉-2来测量来自5名杜氏肌营养不良症(DMD)患者和5名对照者的培养骨骼肌细胞及皮肤成纤维细胞中的细胞内游离钙([Ca++]i)。我们观察到,在静息状态及乙酰胆碱(ACh)刺激后,DMD肌肉细胞中的[Ca++]i水平升高,但未检测到DMD皮肤成纤维细胞与正常皮肤成纤维细胞之间存在任何差异。ACh诱导的异常高的[Ca++]i瞬变表明其在肌肉退化中起关键作用。皮肤成纤维细胞的结果表明不存在普遍的膜缺陷。

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