Effects of succinylacetone on amino acid uptake in the rat kidney.

Infants with hereditary tyrosinemia also have a renal Fanconi syndrome and excrete succinylacetone (SA). We have studied the effects of SA on rat renal tubular amino acid transport in vivo and in vitro using isolated renal tubules. Injection of SA produces increased clearance of several amino acids in the intact animal. In vitro SA causes a reversible inhibition of alpha-aminoisobutyric acid uptake, resulting from depressed low- and high-affinity transport systems. Addition of glutamate, succinate, or glucose, alone or in combination, did not restore transport. These observations suggest the usefulness of SA in the production of a physiologic animal model for the study of the human Fanconi syndrome.