Triheptanoin: long-term effects in the very long-chain acyl-CoA dehydrogenase-deficient mouse.

ABSTRACT: A rather new approach in the treatment of long-chain fatty acid oxidation disorders is represented by triheptanoin, a triglyceride with three medium-odd-chain heptanoic acids (C7), due to its anaplerotic potential. We here investigate the effects of a 1-year triheptanoin-based diet on the clinical phenotype of very long-chain-acyl-CoA-dehydrogenase-deficient (VLCAD^-/-) mice. The cardiac function was assessed in VLCAD^-/- mice by in vivo MRI. Metabolic adaptations were identified by the expression of genes regulating energy metabolism and anaplerotic processes using real-time PCR, and the results were correlated with the measurement of the glycolytic enzymes pyruvate dehydrogenase and pyruvate kinase. Finally, the intrahepatic lipid accumulation and oxidative stress in

SUBMITTER: Tucci S 

PROVIDER: S-EPMC5234721 | biostudies-literature | 2017 Jan

REPOSITORIES: biostudies-literature