title: Stimulation of ketogenesis by propionate in isolated rat hepatocytes: an explanation for ketosis associated with propionic acidaemia and methylmalonic acidaemia?
abstract: The effect of propionate on ketone body production from oleate and octanoate in isolated rat hepatocytes was studied. Propionate (5 mmol/l) stimulated ketogenesis from oleate and octanoate, although the effect was more pronounced when octanoate was used as substrate. Propionate decreased CO2 production from fatty acids, suggesting that propionate inhibited the oxidation of free fatty acid carbons through the tricarboxylic acid cycle. Our results suggest that propionate enhanced ketogenesis as a consequence of the decrease in the rate of the tricarboxylic acid cycle, caused by propionate and/or its derivatives. The stimulation of ketogenesis caused by propionate is discussed as the possible cause of ketosis associated with propionic acidaemia and methylmalonic acidaemia.
mesh_terms: D000138:Acidosis / Q000201:enzymology*; D000592:Amino Acid Metabolism, Inborn Errors / Q000378:metabolism; D000818:Animals; D002245:Carbon Dioxide / Q000378:metabolism; D002478:Cells, Cultured; D005230:Fatty Acids, Nonesterified / Q000378:metabolism; D005260:Female; D007657:Ketone Bodies / Q000378:metabolism; D007659:Ketones / Q000096:biosynthesis*; D007662:Ketosis / Q000201:enzymology*; D050356:Lipid Metabolism; D008099:Liver / Q000378:metabolism*; D008764:Methylmalonic Acid / Q000097:blood; D011422:Propionates / Q000097:blood / Q000494:pharmacology*; D051381:Rats; D011919:Rats, Inbred Strains
chemical_list: D005230:Fatty Acids, Nonesterified; D007657:Ketone Bodies; D007659:Ketones; D011422:Propionates; D002245:Carbon Dioxide; D008764:Methylmalonic Acid