Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disorder with specific damage of motor neurons leading to progressive muscle weakness. The role of oxidative damage in the neurodegenerative process in SMA has been proposed. Polyphenols, with their antioxidant effects, may be used in SMA patients. In this study, the association between oxidative stress and SMA was examined in fibroblast cell lines derived from two different SMA type I patients, one SMA type I carrier and one healthy subject. Reactive oxygen species content of SMA type I, SMA type I carrier, and healthy fibroblasts was compared, and then the antioxidant effect of curcumin was analyzed. Expression differences of the antioxidant genes of SMA type I and healthy cell lines were evaluated with real-time PCR array before and after curcumin treatment. Collectively, our results led us to consider oxidative stress and neuronal development process together within the pathogenesis of SMA, The use of antioxidants, such as curcumin, as a supplement may be an alternative in SMA therapy.
Keywords : Spinal muscular atrophy, Oxidative stress, Cyclooxygenase-2, Curcumin