BLOOD SUPEROXIDE-DISMUTASE, CATALASE AND GLUTATHIONE-PEROXIDASE ACTIVITIES IN FAMILIAL AND SPORADIC AMYOTROPHIC-LATERAL-SCLEROSIS

Recent studies have implicated free radicals in the pathogenesis of am yotrophic lateral sclerosis (ALS), a fatal, paralytic disorder of moto r neurons. Herein we report on measurements of erythrocyte activity of the three main free radical scavenging enzymes: copper/zinc superoxid e dismutase (Cu/Zn-SOD), catalase, and glutathione peroxidase. We stud ied 31 patients with sporadic ALS, 18 with familial ALS, and 24 contro ls, Mean Cu/Zn-SOD activity was reduced in eight familial ALS patients with mutations of Cu/Zn-SOD but was normal in patients with both fami lial ALS without identified Cu/Zn-SOD mutations and sporadic ALS. Glut athione peroxidase activity was significantly reduced only in sporadic ALS patients treated with insulin-like growth factor I (100 mu g/kg). Catalase activity was normal in sporadic and familial ALS. Neither gl utathione peroxidase nor catalase activities correlated significantly with duration of symptoms or age at onset. Vitamin E, vitamin C, and b eta-carotene did not affect any of the three enzyme activities. These observations indicate that disturbances of catalase and glutathione pe roxidase function are not likely to be central factors in the pathogen esis of ALS. (C) 1996 Academic Press Limited.