PROTECTION OF GRANULOCYTES BY VITAMIN-E IN GLUTATHIONE SYNTHETASE DEFICIENCY

The administration of vitamin E (alpha-to-copherol) was found to improve polymorphonuclear leukocyte function in an infant with congenital deficiency of glutathione synthetase activity. Before therapy with vitamin E the abnormal leukocytes exposed to phagocytic challenge showed oxidant damage. They released 60 per cent more hydrogen peroxide than did normal leukocytes, iodinated only 20 to 25 per cent of the normal number of particles, and were unable to kill bacteria as effectively as normal leukocytes although the rates of phagocytosis were normal. These functional abnormalities disappeared when the patient was placed on 400 IU of alpha-tocopherol daily for three months. Associated with the functional improvement was a normalization of microtubule assembly during phagocytic challenge. (N Engl J Med 301:901–905, 1979) GLUTATHIONE, together with glutathione peroxidase and glutathione reductase, plays an important part in regulating the ability of cells to withstand exposure to oxidative drugs and infections.1 The glutathione peroxidase-glutathione reductase system disposes of hydrogen peroxide by employing reduced glutathione as a substrate for hydrogen peroxide in a reaction catalyzed by glutathione peroxidase: The oxidized glutathione is then reconverted to reduced glutathione by glutathione reductase in which NADP+ is generated: In the red blood cell, the inability to reduce adequately glutathione or to synthesize glutathione leads to hemolytic anemia.1,2 In human polymorphonuclear leukocytes unable to synthesize glutathione, cellular membrane and microtubule. © 1979, Massachusetts Medical Society. All rights reserved.