High activities of erythrocyte glutathione peroxidase in patients with the Lesch-Nyhan syndrome.
Antioxidant levels were determined in five patients with the Lesch-Nyhan syndrome. The erythrocyte glutathione peroxidase activity was in average 1.8 times higher in Lesch-Nyhan patients than in controls (1.68 +/- 0.36 versus 0.92 +/- 0.17 mu kat/g hemoglobin, p less than 0.001). Plasma CuZn-superoxide dismutase activity was two times higher (p less than 0.001) and Mn-superoxide dismutase activity was 1.5 times higher (p less than 0.05) than in controls, whilst erythrocyte CuZn-superoxide dismutase, plasma extracellular-superoxide dismutase and lymphocyte superoxide dismutase did not differ between Lesch-Nyhan patients and healthy controls. These data might indicate that Lesch-Nyhan patients are exposed to a higher oxidative load than healthy control persons.
Saugstad
O D
OD
Department of Pediatrics, National Hospital (Rikshospitalet), Oslo, Norway.
Marklund
S L
SL
D000293:Adolescent; D000328:Adult; D002648:Child; D004912:Erythrocytes / Q000201:enzymology*; D005979:Glutathione Peroxidase / Q000097:blood*; D006801:Humans; D007926:Lesch-Nyhan Syndrome / Q000201:enzymology*; D008214:Lymphocytes / Q000201:enzymology; D013482:Superoxide Dismutase / Q000097:blood
D005979:Glutathione Peroxidase; D013482:Superoxide Dismutase