Amelioration of serum 8-OHdG level by enzyme replacement therapy in patients with Fabry cardiomyopathy

Kuan Hsuan Chen, Yueh Ching Chou, Chen Yuan Hsiao, Yueh Chien, Kang Ling Wang, Ying Hsiu Lai, Yuh Lih Chang, Dau Ming Niu, Wen Chung Yu

Research output: Contribution to journalArticlepeer-review

9 Citations (Scopus)

Abstract

Objectives 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. Methods We measured the serum levels of 8-OHdG in 20 healthy control and 22 patients with Fabry cardiomyopathy before and after ERT. Results 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. Conclusions 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.

Original languageEnglish
Pages (from-to)293-299
Number of pages7
JournalBiochemical and Biophysical Research Communications
Volume486
Issue number2
DOIs
Publication statusPublished - Apr 29 2017
Externally publishedYes

Keywords

  • Cardiomyopathy
  • ERT
  • Fabry disease
  • Oxidative stress

ASJC Scopus subject areas

  • Biophysics
  • Biochemistry
  • Molecular Biology
  • Cell Biology

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