carnitine acyl transferase

Carnitine acyltransferase is an enzyme crucial for the metabolism of fatty acids, particularly their transport into the mitochondria for energy production. Fatty acids are a vital energy source, especially during starvation when glycogen stores are depleted.12

The process involves fatty acids being linked to a coenzyme A molecule, forming fatty acyl coenzyme A. This compound then needs to cross the mitochondrial membrane to be oxidized. Carnitine facilitates this transfer by accepting the acyl group from fatty acyl coenzyme A, forming acyl carnitine. Acyl carnitine can then enter the mitochondrion, where it returns the acyl group to coenzyme A for oxidation.1

Defects in carnitine acyltransferase or carnitine transport can lead to inborn errors of metabolism, impacting the body's ability to generate energy from fats. These disorders can manifest with serious health issues, including hypoglycemia, liver disease, and heart problems.2