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.
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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.)
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Chemical References |
- Amino Acids
- Heptanoates
- Heptanoic Acids
- Tyrosine
- succinylacetone
- Sodium
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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)
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