Turkish Journal of Gastroenterology
Liver - Original Article

Investigation of Dynamic Thiol/Disulfide Homeostasis and Nitrosative Stress in Patients with Wilson Disease


Department of Gastroenterology, Faculty of Medicine, Gaziantep University, Gaziantep, Turkey


Department of Medical Pharmacology, Faculty of Medicine, Gaziantep University, Gaziantep, Turkey


Department of Physiology, Faculty of Medicine, Gaziantep University, Gaziantep, Turkey


Medical Student, Faculty of Medicine, Gaziantep University, Gaziantep, Turkey


Department of Neurology, Faculty of Medicine, Istanbul Rumeli University, Istanbul, Turkey


Vocational School of Health Services, Gaziantep University, Gaziantep, Turkey

Turk J Gastroenterol 2021; 32: 765-773
DOI: 10.5152/tjg.2021.20549
Read: 228 Downloads: 101 Published: 01 September 2021

Background: Wilson disease (WD) is an autosomal recessive inherited disorder of copper (Cu2+) metabolism, resulting in Cu2+ accumulation and liver and central nervous system toxicity. Oxidative stress may have a role in the pathogenesis of Wilson disease, but the roles of thiol/disulfide homeostasis and nitrosative stress have not been examined. The purpose of this study was to evaluate whether there is a modification in thiol/disulfide homeostasis and nitrosative stress in patients with Wilson disease.

Methods: A total of 50 patients with Wilson disease (42 under drug treatment and 8 newly diagnosed patients with no drug treatment) and 50 healthy gender- and age-matched controls were enrolled for this study. Serum native thiol and total thiol levels were measured with a spectrophotometric method. The number of disulfide bonds and the related ratios were determined from these measurements. Serum nitric oxide (NO) and 3-nitrotyrosine (3-NT) levels were analyzed using chemiluminescence and ELISA assays, respectively.

Results: The average native thiol levels of the patient group under drug treatment were found to be markedly higher than the levels of controls (P < .05). We detected no marked changes in total thiol and disulfide levels, and disulfide/total thiol, disulfide/native thiol, or
native thiol/total thiol ratios between groups. We found significant elevations in NO levels in Wilson disease group before drug treatment, and the 3-NT levels in the Wilson disease groups prior to (P < .05) and under drug treatment (P < .01), when compared to controls.

Conclusion: Our data are the first to show that nitrosative stress and thiol/disulfide homeostasis can contribute to the pathogenesis of Wilson disease.

Cite this article as: Yücel EM, Konduk BT, Saracaloglu A, et al. Investigation of dynamic thiol/disulfide homeostasis and nitrosative stress in patients with wilson disease. Turk J Gastroenterol. 2021; 32(9): 765-773.

EISSN 2148-5607