title: Protection of granulocytes by vitamin E in glutathione synthetase deficiency.
abstract: The administration of vitamin E (alpha-tocopherol) 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.
mesh_terms: D001770:Blood Bactericidal Activity / Q000187:drug effects; D005981:Glutathione Synthase / Q000172:deficiency*; D006098:Granulocytes / Q000187:drug effects* / Q000502:physiology / Q000648:ultrastructure; D006801:Humans; D006861:Hydrogen Peroxide / Q000097:blood; D007223:Infant; D007231:Infant, Newborn; D007455:Iodine / Q000097:blood; D008297:Male; D010084:Oxidation-Reduction; D010453:Peptide Synthases / Q000172:deficiency*; D010587:Phagocytosis / Q000187:drug effects; D014810:Vitamin E / Q000494:pharmacology / Q000627:therapeutic use*
chemical_list: D014810:Vitamin E; D007455:Iodine; D006861:Hydrogen Peroxide; D010453:Peptide Synthases; D005981:Glutathione Synthase