The Relationship Between The Levels Of Serum Oxide And Antioxide In Patients With LHON And Asympto -matic Carriers Of T

Leber's hereditary optic neuropathy(LHON) is a maternally inherited disease characterized by acute or subacute bilateral visual loss in young patients.the primary actiological event in China is a mutation in the mitochondrial genome ,at bp 11778.lt affect in most cases mtDNA encoded subunits of the respiratory chain NADA:coenzyme Q oxidoreductase(complex I ).The impaired function of complex I leads to a decline in mitochondrial energy production and enhances free radical gener-ation.Objectives: To establish a sensitive and reliable method to detect the 11778 mtDNA mutation ,and to study the levels of serum oxide and antioxide in patients with LHON and asymptomatic carriers of the 11778 mtDNA mutation.Methods: We use allele-specific amplification to detact the 11778 mtDNA mutation,and investigated the serum levels of NOMDA SOD GSH GST and Vit-e in 12 patients with LHON ,17 asymptomatic carriers and 16 controls,using the sepectrophotometric methods.Results: Allele-specific amplification can detect the 11778 mtDNA mutation for LHON,and the serum levels of SOD both in the LHON patients and asymptomatic carr-iers were significantly higher than those of controls (p<0.05), the serum levels of GSH in the patients were significantly higher than those of controls (p < 0.01), while the serum levels of GST and GSH in the asymptomatic carriers were significantly lower than those of controls (p< 0.01 ,p<0.05);As compared with the asympto-matic carriers, the serum levels of NOS in the LHON patients were significantly lower (p<0.01), however,there were no significant difference in the serum levels of MDA NO Vit-e among the three groups.Conclusion: allele-specific amplification was a sensitive and reliable method to detect the 11778 muta-tion for LHON.there was metabolism disorder of free radicals in the LHON patients and asymptomatic carriers.