carnitineshuttlen
The standard term for the system is the carnitine shuttle, a biochemical mechanism that enables long-chain fatty acids to enter the mitochondrial matrix for beta-oxidation. The combined form "carnitineshuttlen" is not commonly used in scientific literature. The shuttle consists of carnitine, the outer and inner mitochondrial membrane enzymes CPT1 and CPT2, and the carnitine-acylcarnitine translocase (CACT), and it is essential for efficient fatty acid utilization in energy metabolism.
Mechanism: In the cytosol, fatty acids are activated to acyl-CoA by acyl-CoA synthetase. CPT1 on the outer
Regulation and significance: CPT1 activity is inhibited by malonyl-CoA, integrating fatty acid oxidation with carbohydrate metabolism.
Clinical notes: Genetic defects include CPT1 deficiency, CPT2 deficiency, and primary carnitine transporter deficiency (SLC22A5). Clinical