Mongini T, Ghigo D, Doriguzzi C, Bussolino F, Pescarmona G, Pollo B, Schiffer D, Bosia A
Clinica Neurologica II, Università degli Studi, Torino, Italy.
Neurology. 1988 Mar;38(3):476-80. doi: 10.1212/wnl.38.3.476.
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.
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。
