Chen Kuan-Hsuan, Chou Yueh-Ching, Hsiao Chen-Yuan, Chien Yueh, Wang Kang-Ling, Lai Ying-Hsiu, Chang Yuh-Lih, Niu Dau-Ming, Yu Wen-Chung
Institute of Clinical Medicine, National Yang-Ming University, Taipei, Taiwan, ROC; Department of Pharmacy, Taipei, Veterans General Hospital, Taipei, Taiwan, ROC.
Institute of Pharmacology, National Yang-Ming University, Taipei, Taiwan, ROC; Department of Pharmacy, Taipei, Veterans General Hospital, Taipei, Taiwan, ROC; School of Pharmacy, Taipei Medical University, Taipei, Taiwan, ROC.
Biochem Biophys Res Commun. 2017 Apr 29;486(2):293-299. doi: 10.1016/j.bbrc.2017.03.030. Epub 2017 Mar 12.
The level of 8-hydroxy-2-deoxyguanosise (8-OHdG) is a marker of oxidative stress. The objective of this study was to evaluate the effect of enzyme replacement therapy (ERT) on the level of 8-OHdG in patients with Fabry cardiomyopathy and the clinical evolution of Fabry cardiomyopathy.
We measured the serum levels of 8-OHdG in 20 healthy control and 22 patients with Fabry cardiomyopathy before and after ERT.
The mean lysoGb3 and 8-OHdG levels was significantly increased in patients with Fabry cardiomyopathy compared with that of control subjects (lysoGb3, 3.6 ± 1.1 nM vs. 0.4 ± 0.1 nM, p < 0.01; 8-OHdG, 4.5 ± 0.5 ng/mL vs. 3.4 ± 0.4 ng/mL, P < 0.05). The mean lysoGb3 and 8-OHdG levels was significantly reduced after ERT for 14.2 months (lysoGb3, 3.6 ± 1.1 nM vs. 2.9 ± 1.1 nM, P < 0.05; 8-OHdG, 4.5 ± 0.5 ng/mL to 4 ± 0.4 ng/mL, P < 0.05). These changes were accompanied by decreases in LVM and LVMI.
We demonstrated that the serum 8-OHdG levels is increased in patients with Fabry cardiomyopathy (FC) and that successful management of FC with ERT is associated with a decrease in this oxidative stress marker. Serum 8-OHdG levels can be used not only as a noninvasive biomarker of oxidative stress in patients with FC but also an objective and quantitative parameter in the follow-up of patients during ERT.
8-羟基-2-脱氧鸟苷（8-OHdG）水平是氧化应激的一个标志物。本研究的目的是评估酶替代疗法（ERT）对法布里心肌病患者8-OHdG水平及法布里心肌病临床进展的影响。
我们测量了20名健康对照者和22名法布里心肌病患者在ERT治疗前后的血清8-OHdG水平。
与对照受试者相比，法布里心肌病患者的平均溶酶体Gb3和8-OHdG水平显著升高（溶酶体Gb3，3.6±1.1 nM对0.4±0.1 nM，p<0.01；8-OHdG，4.5±0.5 ng/mL对3.4±0.4 ng/mL，P<0.05）。ERT治疗14.2个月后，平均溶酶体Gb3和8-OHdG水平显著降低（溶酶体Gb3，3.6±1.1 nM对2.9±1.1 nM，P<0.05；8-OHdG，4.5±0.5 ng/mL降至4±0.4 ng/mL，P<0.05）。这些变化伴随着左心室质量（LVM）和左心室质量指数（LVMI）的降低。
我们证明法布里心肌病（FC）患者血清8-OHdG水平升高，并且ERT成功治疗FC与这种氧化应激标志物的降低有关。血清8-OHdG水平不仅可以用作FC患者氧化应激的非侵入性生物标志物，还可以作为ERT期间患者随访的客观定量参数。
