Thiol/disulfide homeostasis as a new oxidative stress marker in patients with Fabry disease

Abstract

This is the first study to evaluate thiol-disulfide homeostasis and oxidative stress (OS) levels in patients with Fabry Disease (FD). This prospective study consists of 30 FD patients and 30 healthy controls. The dynamic thiol-disulfide homeostasis of patients and control groups was evaluated by using the new, cost-effective and fully automated colorimetric method. A total of 60 patients and controls were included the present study. A statistically significant difference was found between the patient and control groups for native and total thiol levels (p<0.001). In addition, disulfide levels were significantly higher in FD patients compared with the control group (p<0.003). Native thiol levels showed significanlty negative correlation with Lyso-Gb3, total oxidant status (TOS) and oxidative stress index (OSI) levels. In addition, a positive correlation was found between disulfide/natural thiol and disulfide/total thiol ratios and TOS, OSI and BUN. We found total antioxidant status (TAS) levels were lower in the patient group compared to the control group, while TOS and OSI levels were higher and were statistically significant. This study highlights for the first time a novel, cost-effective and fully automated measurement of thiol -disulfide levels in patients with FD. Dynamic thiol- disulfide levels may contribute to the etiopathogenesis and follow-up of the disease in patients with FD.

Effect of mode of delivery on neonatal oxidative stress and dynamic thiol–disulfide homeostasis

“…This increased oxygen consumption leads to an increase in the production of free radicals, causing OS. 12,25,26 Thiols are composed of sulfur and hydrogen atoms attached to a carbon atom, and play a significant role in the elimination of OS in cells. 27 When exposed to oxidation, thiols transform into disulfide structures, which is an indication of an early cellular response to OS.…”

“…This situation plays an important role in the pathogenesis of many diseases. 12,13 Thiols, which contain a sulfhydryl group attached to the carbon atom in their structure, are an important part of the antioxidant system. During OS, thiols undergo oxidation reactions to form disulfide structures, 4,14 which are then reduced back to thiol groups to achieve a thiol-disulfide balance.…”