Abstract
Extract: Propionic acid significantly inhibited 14CO2 production from [1-14C]palmitate at a concentration of 10 μM in control fibroblasts and 100 μM in methylmalonic fibroblasts. This inhibition was similar to that produced by 4-pentenoic acid. Methylmalonic acid also inhibited 14CO2 production from [1-14C]palmitate, but only at a concentration of 1 mM in control cells and 5 mM in methylmalonic cells.
Propionic acid (5 mM) also inhibited ureagenesis in rat liver slices when ammonia was the substrate but not with aspartate and citrulline as substrates. Propionic acid had no direct effect on either carbamyl phosphate synthetase or ornithine transcarbamylase.
These findings may explain the fatty degeneration of the liver and the hyperammonemia in propionic and methylmalonic acidemia.
Speculation: It has been shown that 4-pentenoic acid will produce may of the features of Reye's syndrome in rats. The fact that propionic acid inhibits some of the same reactions as 4-pentenoic acid raises the possibility that other short chain fatty acids less unusual than 4-pentenoic acid could produce the features of Reye's syndrome.
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Glasgow, A., Chase, P. Effect of Propionic Acid on Fatty Acid Oxidation and Ureagenesis. Pediatr Res 10, 683–686 (1976). https://doi.org/10.1203/00006450-197607000-00010
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DOI: https://doi.org/10.1203/00006450-197607000-00010
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