Mitochondrial Superoxide Dismutase Activity in Amyotrophic Lateral Sclerosis

Background: Evidence suggests that mitochondrial dysfunction and oxidative stress may be involved in the pathogenesis of Amyotrophic Lateral Sclerosis (ALS). Some studies show the presence of altered anti-oxidative defence enzyme activity in the blood of ALS patients. It has also been demonstrated that a superoxide-dismutase-1 (SOD1) enzyme fraction is located in the mitochondria. Objective: To Study the correlation between SOD1 activity and clinical data in ALS-patients. Patients and Methods: SOD1, SOD2 and aconitase activity was analysed in mitochondria of platelets from nine sporadic- ALS (SALS) patients and nine healthy-controls. The biochemical data was compared and correlated with clinical data from the ALS-patients. Results: Mitochondrial SOD1 activity was lower in ALS patients than in healthy controls, and much lower in more serious cases of ALS associated with respiratory insufficiency, weight-loss, low scores on the ALSFRS scale and the elderly. Conclusion: Mitochondrial SOD1 activity may be involved in the clinical evolution of SALS.