Zinc Levels and Oxidative Stress in Ataxia-telangiectasia Patients

RATIONALE: Ataxia-Telangiectasia (AT) is a rare degenerative autosomal recessive disease caused by a mutation in the gene encoding ATM protein (11q22-23). These patients tend to present increased oxidative stress and also neurodegeneration and a predisposition to cancer . There aren't any studies evaluating the role of antioxidant nutrients as zinc in the exacerbation of oxidative stress. OBJECTIVE: To compare lipid peroxidation and zinc levels in AT patients to healthy controls. METHODS: Fourteen AT patients from the Immunology outpatients clinic at the Federal University of Sao Paulo and age and gender-matched healthy controls underwent laboratorial evaluation for erythrocyte zinc and plasma malondialdehyde (marker of oxidative stress). RESULTS: Patients and controls mean age was 13.2 ± 4.8 years and 78.6% (11/14) were males. Mean erythrocyte zinc levels were 43.3 ±9.8 μgZn/Hb for patients and 44.8 ±8.3 μgZn/Hb for controls, p=0.66. Mean levels of malondialdehyde were 3.12 ± 0.7 nmol/mL and 2.6 ± 0.5 nmol/mL, for patients and controls respectively. High levels of malondialdehyde were observed only in four patients, but there was no significant difference between the two groups (p=0,06). No correlation was found between malondialdehyde and erythrocyte zinc levels in either group (p=0.34). CONCLUSIONS: AT patients have a tendency to have high levels of malondialdehyde which suggest an increase oxidative stress.