Increased mitochondrial oxidative damage in patients with sporadic amyotrophic lateral sclerosis

To investigate whether mitochondrial oxidative damage contributes to the pathogenesis of sporadic amyotrophic lateral sclerosis (sALS), we used high-performance liquid chromatography with an electrochemical detector to measure the concentrations of the reduced and oxidized forms of coenzyme Q10 (CoQ10) in the cerebrospinal fluid (CSF) of 30 patients with sALS and 17 age-matched controls with no neurological diseases.The percentage of oxidized CoQ10 in the CSF of sALS patients were significantly greater than those in the CSF of controls (P < 0.002) and were negatively correlated with duration of illness (ρ = − 0.64, P < 0.001).These results suggest that mitochondrial oxidative damage contributes to the pathogenesis of sporadic amyotrophic lateral sclerosis.