A study of subject with erythrocyte glucose-6-phosphate dehydrogenase deficiency. II. Investigation of leukocyte enzymes

A hereditary abnormality of the erythrocytes was described in Negroes sensitive to primaquine (1). A similar or identical defect has been de-tected in the erythrocytes of a considerable pro-portion of non-Ashkenazic Jews susceptible to favism and sensitive to various drugs (2-4). The primary defect of these erythrocytes is prob-ably the markedly decreased activity of glucose-6-phosphate dehydrogenase (5). The cells, how-ever, demonstrate a multitude of secondary ab-normalities, namely: a low glutathione (GSH) level; glutathione instability; a decreased glycine incorporation rate into GSH in vitro; and an in-crease in glutathione reductase, aldolase and tri-phosphopyridine nucleotide (6-9). This hered