Year: 2011
Type: article
Abstract: The dietary treatment of patients with long-chain fatty acid oxidation disorders is in a transition period from classical medium-even-chain trioctanoin to mediumodd-chain triheptanoin.In my first project, I investigated the interrelations between C 4 -ketogenesis (production of β-hydroxybutyrate + acetoacetate), C 5ketogenesis (production of β-hydroxypentanoate + ketopentanoate), and anaplerosis in isolated rat livers perfused with 13 C-labeled octanoate, heptanoate, or propionate.Although the uptakes of octanoate and heptanoate by the liver are similar, the rate of C 5 -ketogenesis from heptanoate is much lower than the rate of C 4 -ketogenesis.This results from the channeling of the propionyl moiety of heptanoate into anaplerosis and gluconeogenesis.C 5 -ketogenesis from propionate is virtually nil because the kinetics of acetoacetyl-CoA thiolase do not favor the formation of β-ketopentanoyl-CoA from propionyl-CoA and acetyl-CoA.Anaplerosis and gluconeogenesis from heptanoate are inhibited by octanoate.The data have implications for the design of diets for the treatment of long-chain fatty acid oxidation disorders, such as the triheptanoin-based diet.
Author: Shuang Deng
Institution: Case Western Reserve University
Language: English
Subfield: Clinical Biochemistry
Domain: Life Sciences
Open Access status: green
Funder: National Institutes of Health
OpenAlex ID: W2222610974