OBJECTIVE: To determine the serum levels of total superoxide dismutase (SOD) activity and malondialdehyde (MDA), and to evaluate the oxidant-antioxidant status in patients with Leber's hereditary optic neuropathy (LHON) carrying the mitochondrial G11778A mutation. METHODS: Nineteen patients and 12 carriers from three Chinese G11778A LHON families were enrolled in this study, and 30 age-matched healthy volunteers were recruited as normal controls. The serum levels of total SOD activity and MDA in all subjects were measured by xanthine oxidase test and thiobarbituric acid technique, respectively. RESULTS: The serum level of total SOD activity in LHON patients was significantly less than that in carriers and normal controls (q = 7.085 and 8.351, respectively, both P < 0.05), however, there was no significant difference between the carriers and normal controls (q = 0.269, P > 0.05). The serum level of MDA in patients and carriers was significantly higher than that in normal controls (q = 9.069 and 4.748, respectively, both P < 0.05), and it was also significantly higher in patients than that in carriers (q = 3.618, P < 0.05). CONCLUSIONS: Antioxidant capacity decreased significantly in patients with LHON, which indicates that the onset of LHON was related with the oxidation-antioxidation imbalance.
OBJECTIVE: To determine the serum levels of total superoxide dismutase (SOD) activity and malondialdehyde (MDA), and to evaluate the oxidant-antioxidant status in patients with Leber's hereditary optic neuropathy (LHON) carrying the mitochondrial G11778A mutation. METHODS: Nineteen patients and 12 carriers from three Chinese G11778A LHON families were enrolled in this study, and 30 age-matched healthy volunteers were recruited as normal controls. The serum levels of total SOD activity and MDA in all subjects were measured by xanthine oxidase test and thiobarbituric acid technique, respectively. RESULTS: The serum level of total SOD activity in LHON patients was significantly less than that in carriers and normal controls (q = 7.085 and 8.351, respectively, both P < 0.05), however, there was no significant difference between the carriers and normal controls (q = 0.269, P > 0.05). The serum level of MDA in patients and carriers was significantly higher than that in normal controls (q = 9.069 and 4.748, respectively, both P < 0.05), and it was also significantly higher in patients than that in carriers (q = 3.618, P < 0.05). CONCLUSIONS: Antioxidant capacity decreased significantly in patients with LHON, which indicates that the onset of LHON was related with the oxidation-antioxidation imbalance.