Oxidative stress in Amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis (ALS) is a disease of neurodegenerative disorder that is characterized by progressive degeneration of motor neurons and the patients are usually dead within 2-3 years of symptom onset. The causes of ALS are not completely understood yet, while the disease progression is diverse and complex. It is believed that multiple mechanisms underlie the disease progression and oxidative stress is considered to be one of the important factors. About 20% of familial ALS cases are linked to mutation in the gene encoding the superoxide dismutase 1(SOD1). It is found that the markers of oxidative damage are elevated in the spinal cord and cerebrospinal fluid of ALS patients. In this review, we summarize the importance and the source of oxidative stress in ALS pathogenesis. Key words: ALS; Oxidative stress; SOD1; NRF2