抄録
Activities of platelet superoxide dismutase (SOD), catalase and glutathione peroxidase were measured in the family members of the hereditary heterozygous glutathione reductase deficiency. The SOD activity of four deficient individuals fell in the range from 3.62 to 4.28unit/mg protein, significantly high compared to normal subject (2.52±0.26unit/mg protein). The cyanide insensitive SOD (mitochondrial SOD) of the members had not increased compared to that of normal subject, and the increased activity was found to be due to cyanide sensitive SOD (cytoplasmic SOD). On the other hand both catalase and glutathione peroxidase activities of the deficient members were almost within normal range (35.4±7.0μ mole H2O2/min/mg protein for catalase, 1.15±0.23×10-2μ mole NADPH/min/mg protein for glutathione peroxidase). The platelet aggregation of the proband was also examined and found to be normal; aggregation induced with ADP (10-5M), epinephrine (2.6×10-7M), collagen (10μg/ml) or ristocetin (1.5mg/ml). The increased SOD activity might be due to a compensatory effect for the increasing level of active oxygen, that was brought about due to the defect of glutathione system. The compensation might also account for the normal platelet function.
