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

Abstract
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.
AuthorsP D Spencer, K S Roth
JournalBiochemical medicine and metabolic biology (Biochem Med Metab Biol) Vol. 37 Issue 1 Pg. 101-9 (Feb 1987) ISSN: 0885-4505 [Print] United States
PMID3566973 (Publication Type: Journal Article, Research Support, U.S. Gov't, P.H.S.)
Chemical References
  • Amino Acids
  • Aminoisobutyric Acids
  • Heptanoates
  • Heptanoic Acids
  • 2-aminoisobutyric acid
  • succinylacetone
Topics
  • Amino Acids (metabolism)
  • Aminoisobutyric Acids (metabolism)
  • Animals
  • Fanconi Syndrome (metabolism)
  • Heptanoates (pharmacology)
  • Heptanoic Acids (pharmacology)
  • In Vitro Techniques
  • Kidney Tubules (drug effects, metabolism)
  • Male
  • Rats
  • Rats, Inbred Strains

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