Department of Pediatric Neurology, Cukurova University Faculty of Medicine, Toros mah. 78186 sok., Yeşilpark Evleri, Kat: 7, No: 13, Adana, Turkey.
Department of Biochemistry, Ankara Ataturk Training and Research Hospital, Ankara, Turkey.
Acta Neurol Belg. 2019 Jun;119(2):215-218. doi: 10.1007/s13760-018-01072-5. Epub 2019 Jan 3.
Duchenne muscular dystrophy (DMD) is a disorder that alter the expression of the dystrophin protein. Dystrophin deficiency alters the structural integrity of the contractile apparatus/sarcolemmal integrity, leading to dystrophic changes. Dystrophin deficiency results in an increase in oxidative stress. We aimed to investigate the thiol/disulfide balance as an oxidative stress marker in children with DMD. We included 24 DMD, and 22 healthy control group subjects in the study. The total thiol, native thiol, and disulphide levels were measured and the disulphide/native thiol, disulphide/total thiol and native thiol/total thiol ratios were calculated in DMD patients and healthy subjects. The mean age distribution of the patients and the healthy control group subjects was similar. The total thiol, native thiol, and disulfide levels were lower in DMD group than the healthy controls. In conclusion, the markers and ratios were measured and calculated in the blood, and we detected that the total thiol, and native thiol levels were lower in DMD group than the healthy controls. These results indicate that dynamic thiol-disulphide homeostasis can be used as a marker of oxidative stress in clinical trials with DMD.
杜氏肌营养不良症（DMD）是一种改变肌营养不良蛋白表达的疾病。肌营养不良蛋白缺乏会改变收缩装置/肌膜完整性的结构完整性，导致肌营养不良变化。肌营养不良蛋白缺乏会导致氧化应激增加。我们旨在研究硫醇/二硫键平衡作为 DMD 儿童的氧化应激标志物。我们纳入了 24 名 DMD 患者和 22 名健康对照组受试者进行研究。在 DMD 患者和健康受试者中测量了总巯基、天然巯基和二硫键水平，并计算了二硫键/天然巯基、二硫键/总巯基和天然巯基/总巯基的比值。患者和健康对照组受试者的平均年龄分布相似。DMD 组的总巯基、天然巯基和二硫键水平均低于健康对照组。总之，我们在血液中测量并计算了这些标志物和比值，发现 DMD 组的总巯基和天然巯基水平均低于健康对照组。这些结果表明，动态硫醇-二硫键平衡可以作为 DMD 临床试验中氧化应激的标志物。
