Abstract Long-chain fatty acid oxidation disorders (LC-FAODs) represent a group of rare inborn errors of metabolism characterized by acute crises of energy metabolism and severe energy deficiency.

Impaired mitochondrial fatty acid beta-oxidation is primarily responsible for reduced fatty acid degradation, which disrupts energy balance and lipid homeostasis in the body.

By thoroughly characterizing mutants defective in SUGAR-DEPENDENT1 (SDP1) triacylglycerol lipase or PEROXISOMAL ABC TRANSPORTER 1 (PXA1), here we show that TAG is a key intermediate in the ...

Inducible regulatory T (iTreg) cells play a crucial role in immune suppression and are important for the maintenance of immune homeostasis. Mounting evidence has demonstrated connections between iTreg ...

The FDA has approved Dojolvi™ (triheptanoin; Ultragenyx) as a source of calories and fatty acids for the treatment of pediatric and adult patients with molecularly confirmed long-chain fatty ...

The MCU protein is a channel to bring calcium into the mitochondria, and Cpt1a is an enzyme in mitochondria that is the rate-limiting step for the fatty acid beta-oxidation energy pathway.

In the liver, these medium-chain fatty acids are partly converted into storage fats (triglycerides). Exactly how this happens was largely unknown until now.

* ULTRAGENYX ANNOUNCES U.S. FDA APPROVAL OF DOJOLVI™ (UX007/TRIHEPTANOIN), THE FIRST FDA-APPROVED THERAPY FOR THE TREATMENT OF LONG-CHAIN FATTY ACID OXIDATION DISORDERS ...

The drug promotes lipophagy and hepatic fatty acid beta-oxidation to reduce liver fat, inflammation, and liver fibrosis.