Copper accumulation in tissues due to a biallelic pathogenic mutation of the gene: ATP7B results in a clinical phenotype known as
Wilson disease (WD). Aberrations in
copper homeostasis can create favourable conditions for
superoxide-yielding redox cycling and oxidative tissue damage. Drugs used in WD treatment aim to remove accumulated
copper and normalise the free
copper concentration in the blood. In the current study the effect of decoppering treatment on
copper metabolism and systemic
antioxidant capacity parameters was analyzed. Treatment naïve WD patients (TNWD) (n = 33), those treated with anti-
copper drugs (TWD) (n = 99), and healthy controls (n = 99) were studied. Both TNWD and TWD patients characterised with decreased
copper metabolism parameters, as well as decreased total
antioxidant potential (AOP),
glutathione (GSH) level, activity of
catalase,
glutathione peroxidase (GPx), and
S-transferase glutathione, compared to controls. TWD patients had significantly lower
copper metabolism parameters, higher total AOP and higher levels of GSH than TWD individuals; however, no difference was observed between these two patient groups with respect to the rest of the
antioxidant capacity parameters. Patients who had undergone treatment with
D-penicillamine or
zinc sulphate did not differ with respect to
copper metabolism or
antioxidant capacity parameters, with the exception of GPx that was lower in
D-penicillamine treated individuals. These data suggest that anti-
copper treatment affects
copper metabolism as well as improves, but does not normalize, natural
antioxidant capacity in patients with WD. We propose to undertake studies aimed to evaluate the usefulness of
antioxidants as well as
selenium as a supplemental
therapy in WD.