Carnitine palmitoyltransferase

Function Carnitine palmitoyltransferase (CPT I and CPT II) are involved in the transport of long-chain fatty acids into the mitochondria where they are oxidized. Fatty acids form a conjugate with CoA before being oxidized in the mitochondria. However, the CoA-long-chain fatty acid conjugates can not diffuse into the mitochondria. CPT I is a membrane protein which substitutes the CoA in the long-chain fatty acids by carnitine. After entering the mitochondria. CPT II exchanges the carnitine by CoA, enabling the oxidation of the long-chain fatty acids. See also Beta oxidation. Disease CPT I deficiency prevents the body from using certain fats for energy, particularly during fasting. It is associated with encephalopathy, seizures and unexpected infancy death. CPT I is important in fatty acid disorders like diabetes. Structural highlights (2rcu) in a with its situated at the tunnel center and the of the tunnel.

spinqualitylabelsall models Rat carnitine palmitoyltransferase II dimer complex with substrate analog and octylglucoside (PDB code 2rcu) Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image

3D structures of carnitine palmitoyltransferase3D structures of carnitine palmitoyltransferase

Updated on 16-January-2024

2le3 – hCPT I regulatory domain – human - NMR
2m76 - hCPT I regulatory domain – NMR
2h4t, 2fyo, 2deb – rCPT II – rat
2fw3 - rCPT II + antidiabetic drug
2rcu – rCPT II + substrate analog
4ep9, 4eph, 4eyw - rCPT II + inhibitor