Glutathione metabolism in canine hereditary stomatocytosis with mild erythrocyte glutathione deficiency.

Mild glutathione deficiency can accompany hereditary stomatocytosis in man and dogs. We studied several facets of glutathione metabolism in dogs to better define hereditary stomatocytosis. In vivo glutathione flux was measured with alpha-ketoglutarate-alpha-(2-14C) and glycine-(2-3H) in a double-labeled experiment. Glycine-labeled glutathione turnover was faster than that of alpha-ketoglutarate-labeled glutathione, and the glutathione flux was greater in HSt cells than in normal cells. The glutathione-reducing enzymes, glutathione reduction rate, glutathione precursors (except cysteine), oxidized glutathione percentage, and erythrocyte water were increased significantly in HSt cells. Glutathione synthetic enzymes, oxidized glutathione transport system, and glutathione-S-transferase did not differ significantly. These observations suggest that the mild glutathione deficiency in canine hereditary stomatocytosis is a reflection of increased catabolism.