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杜兴氏肌营养不良症携带者的肌核钙。一项X射线微量分析研究。

Myonuclear calcium in carriers of Duchenne muscular dystrophy. An X-ray microanalysis study.

作者信息

Maunder-Sewry C A, Dubowitz V

出版信息

J Neurol Sci. 1979 Aug;42(3):337-47. doi: 10.1016/0022-510x(79)90167-9.

Abstract

Electron-microscopic X-ray microanalysis (EMMA) was used to study calcium and phosphorus concentrations in the muscle fibre nuclei of 3 genetically definite, 5 probable and 2 possible carriers of Duchenne muscular dystrophy. All cases showed elevated mean calcium:phosphorus ratios compared with 4 female controls and, with the exception of one possible carrier, all showed elevated calcium concentrations. A significantly greater variation of values was also found for both parameters. Analyses were made of nuclei from fibres showing no morphological abnormalities and the results suggest elevation of myonuclear calcium may be an early defect in dystrophic muscle.

摘要

采用电子显微镜X射线微量分析(EMMA)技术,对3例基因明确、5例可能及2例疑似杜兴氏肌营养不良症携带者的肌纤维细胞核中的钙和磷浓度进行了研究。与4名女性对照相比,所有病例的平均钙磷比均升高,除1例疑似携带者外,所有病例的钙浓度均升高。这两个参数的值也存在显著更大的变异性。对无形态学异常的纤维细胞核进行了分析,结果表明肌细胞核钙升高可能是营养不良性肌肉的早期缺陷。

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