Newman R J
Metabolism. 1985 Aug;34(8):737-40. doi: 10.1016/0026-0495(85)90024-1.
The forearm flexor muscles of five patients with Becker's dystrophy were examined by the painless and noninvasive technique of high resolution phosphorus nuclear magnetic resonance spectroscopy. In the mildly affected cases, the ratios of the signals of phosphocreatine to ATP and to inorganic phosphate were normal but they were reduced in the patients with advanced disease. Absolute quantitation under the conditions of the study was not feasible, but it was probable that whereas in advanced Becker's dystrophy the intramyocellular concentration of phosphocreatine was reduced, that of ATP was unchanged. The intramyocellular pH was normal in three of the four patients in whom this could be measured and an additional unidentified signal between those of phosphocreatine and inorganic phosphate was recorded in two patients. This study emphasizes some metabolic similarities between Becker's and Duchenne type muscular dystrophy and suggests that nuclear magnetic resonance spectroscopy may be a useful and objective technique with which to investigate the biochemistry of these and other muscle diseases.
采用高分辨率磷核磁共振波谱这种无痛且非侵入性的技术,对5例贝克型肌营养不良患者的前臂屈肌进行了检查。在病情较轻的病例中,磷酸肌酸与三磷酸腺苷(ATP)以及与无机磷酸盐的信号比值正常,但在病情严重的患者中这些比值降低。在本研究条件下进行绝对定量是不可行的,但很可能在晚期贝克型肌营养不良中,肌细胞内磷酸肌酸浓度降低,而ATP浓度未变。在4例可测量此指标的患者中,有3例的肌细胞内pH值正常,并且在2例患者中记录到了一个位于磷酸肌酸和无机磷酸盐信号之间的额外未识别信号。这项研究强调了贝克型和杜兴型肌营养不良之间的一些代谢相似性,并表明核磁共振波谱可能是一种用于研究这些及其他肌肉疾病生物化学的有用且客观的技术。