Our studies have concerned the metabolic errors of glutathione synthesis and degradation (glutathionuria, glutathione synthase deficiency and gamma glutamyl-cysteine synthase deficiency), glucose-6-phosphate-dehydrogenase deficiency in which the capacity for glutathione reduction is decreased, cystinosis, homocystinuria, phenylketonuria, and non-ketotic hyperglycinemia. As detailed in the attached description of our major findings, we have been interested in the development of newer forms of treatment and diagnosis for a number of these metabolic disorders. In particular we have been evaluating from a therapeutic point of view reducing agents such as ascorbic acid and cysteamine in the treatment of cystinosis, the use of the anti-oxidant vitamin E in the treatment of the abnormalities of glutathione metabolism, and strychnine in the treatment of non-ketotic hyperglycinemia. We have also studied in detailed mechanisms involved in the abnormalities of cellular function associated with the above names inborn errors of metabolism.