Effects of succinylacetone on the uptake of sugars and amino acids by brush border vesicles.

Abstract

Infants with hereditary tyrosinemia excrete succinylacetone (SA) in their urine, and suffer from a reversible renal Fanconi syndrome with glycosuria and hyperaminoaciduria. Thus, we have examined the effects of 4 mM SA on rat renal brush border membrane vesicle uptake of sugars and amino acids. SA, unlike sodium maleate, significantly inhibits Na+-dependent vesicular sugar and amino acid uptake. 22Na-uptake, as well as membrane fluidity of the vesicles, are also affected by SA. Inhibition of glycine uptake by SA is reversible and competitive in nature, while alpha-CH3-D-glucoside uptake is non-competitively affected. We conclude, therefore, that SA has a more complex action on the rat renal tubule than sodium maleate, and is likely a much more physiologic model for study of the human renal Fanconi syndrome.

• Authors: P D Spencer, M S Medow, L C Moses, K S Roth
• Journal: Kidney international (Kidney Int) Vol. 34 Issue 5 Pg. 671-7 (Nov 1988) ISSN: 0085-2538 [Print] United States
• PMID: 3199678 (Publication Type: Journal Article, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Amino Acids
• Heptanoates
• Heptanoic Acids
• Tyrosine
• succinylacetone
• Sodium

Topics

• Amino Acid Metabolism, Inborn Errors (metabolism)
• Amino Acids (metabolism)
• Animals
• Biological Transport
• Carbohydrate Metabolism
• Fanconi Syndrome (metabolism)
• Heptanoates (pharmacology)
• Heptanoic Acids (pharmacology)
• Kidney Tubules, Proximal (metabolism)
• Male
• Microvilli (metabolism)
• Rats
• Rats, Inbred Strains
• Sodium (metabolism)
• Tyrosine (blood)