Grosso Salvatore, Perrone Serafina, Longini Mariangela, Bruno Carlo, Minetti Claudio, Gazzolo Diego, Balestri Paolo, Buonocore Giuseppe
Department of Pediatrics, Obstetrics and Reproductive Medicine, Viale Mario Bracci 36, 53100 Siena, Italy.
Brain Dev. 2008 Jun;30(6):391-5. doi: 10.1016/j.braindev.2007.11.005. Epub 2008 Jan 3.
Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are degenerative disorders of muscle. Although the mechanisms underlying muscle degeneration are still uncertain, oxidative-damage has been proposed to play a key role. Isoprostanes are markers of free radical-catalyzed lipid peroxidation; the aim of our study was to evaluate plasma isoprostane levels in group of patients affected by Duchenne and Becker muscular dystrophies. PF(2)-isoprostane levels were measured by colorimetric enzyme immunoassay in the plasma of 17 patients with DMD and 24 with BMD. When compared to a group of healthy controls, affected patients showed significantly higher plasma levels of isoprostanes (p=0.001). When patients were stratified according to the clinical diagnosis, isoprostane levels were not statistically different between DMD and BMD patients. In conclusion whether the condition of oxidative stress found in plasma depends on the degenerative process occurring in muscles or on different mechanisms, such as the release of myoglobin in the blood, should be ascertained. However, our study confirms that oxidative stress findings in DMD/BMD patients are effectively present at the plasma levels. The condition of oxidative stress might act as an adjunctive cause of extra-muscular cell damage to which these patients are exposed for their entire life.
杜兴氏肌营养不良症(DMD)和贝克氏肌营养不良症(BMD)是肌肉的退行性疾病。尽管肌肉退化的潜在机制仍不明确,但有观点认为氧化损伤起关键作用。异前列腺素是自由基催化脂质过氧化的标志物;我们研究的目的是评估杜兴氏和贝克氏肌营养不良症患者组的血浆异前列腺素水平。采用比色酶免疫分析法测定了17例DMD患者和24例BMD患者血浆中的PF(2)-异前列腺素水平。与一组健康对照相比,患病患者的血浆异前列腺素水平显著更高(p = 0.001)。当根据临床诊断对患者进行分层时,DMD和BMD患者的异前列腺素水平在统计学上没有差异。总之,血浆中发现的氧化应激状态是取决于肌肉中发生的退行性过程还是取决于不同机制,如肌红蛋白释放入血,这一点尚需确定。然而,我们的研究证实DMD/BMD患者血浆水平确实存在氧化应激。氧化应激状态可能是这些患者终生面临的肌肉外细胞损伤的一个辅助原因。