Therapeutic effects of glycine in isovaleric acidemia

Abstract

Leucine, which is not normally metabolized in isovaleric acidemia, was given to a patient with this disorder as a single amino acid load and in the form of a protein meal, first alone and then in conjunction with oral glycine. It was shown that glycine has a detoxifying effect because it decreased the level of serum isovaleric acid and caused an increased urinary excretion of the nontoxic conjugation product, isovalerylglycine. Long term glycine administration was well tolerated but did not prevent ketotic episodes induced by infections. During several attacks rectal glycine was effective in shortening the duration of the ketosis.