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Dynamic thiol/disulphide homeostasis in children with Duchenne muscular dystrophy

  • Faruk IncecikEmail author
  • Gamze Avcıoğlu
  • Özcan Erel
  • Salim Neşelioğlu
  • Ozlem M. Hergüner
Original article
  • 29 Downloads

Abstract

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.

Keywords

Oxidative stress Dynamic thiol-disulphide homeostasis Duchenne muscular dystrophy 

Notes

Funding

The authors received no financial support for the research, authorship, and/or publication of this article.

Compliance with ethical standards

Conflict of interest

The authors declared no potential conflict of interest with respect to the research, authorship, and/or publication of this article.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from the parents of the child included in the study.

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Copyright information

© Belgian Neurological Society 2019

Authors and Affiliations

  • Faruk Incecik
    • 1
    Email author
  • Gamze Avcıoğlu
    • 2
  • Özcan Erel
    • 2
  • Salim Neşelioğlu
    • 2
  • Ozlem M. Hergüner
    • 1
  1. 1.Department of Pediatric NeurologyCukurova University Faculty of MedicineAdanaTurkey
  2. 2.Department of BiochemistryAnkara Ataturk Training and Research HospitalAnkaraTurkey

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