Plasma total antioxidant activity in Wilson's disease patients

Wilson's disease is an inherited disorder of copper metabolism characterized by the defective biliary excretion of copper and impairment of its incorporation into ceruloplasmin. The copper-binding protein, ceruloplasmin is one of the plasma antioxidants and prevents lipid peroxidation and generation of free radicals. Serum ceruloplasmin levels are low in most Wilson's disease patients. Since plasma antioxidant system has many components, a deficiency of any component can cause a reduction in the total antioxidant status of an individual. In this study, we investigated whether the low levels of ceruloplasmin cause any reduction in the total antioxidant capacity of plasma in Wilson's disease patients. Plasma total antioxidant activities of 17 Wilson's disease patients (mean age: 11.43 ± 4.8 y.) and of 31 healthy controls (mean age:10.93 ± 3.3 y.) were measured. Of our Wilson's disease patients, 8 had low ceruloplasmin levels (< 20 mg/dl), 9 had normal ceruloplasmin levels (?20 mg/dl). It was used ABTS.* radical cation decolorization assay for determination of antioxidant activity. In this assay, total antioxidant activity was expressed as trolox equivalent antioxidant capacity (TEAC). There was no significant difference between the TEAC values of the controls and of Wilson's disease patients (p>0.05). Furthermore, the TEAC values of the patients with low and normal ceruloplasmin levels were similar to that of the control group.