Carnitine palmitoyltransferase

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   Print3D 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 Substrate analog interacts with CPT II (2rcu) in a large tunnel with its hydrophilic head group situated at the tunnel center and the alkyl part occupying the hydrophobic part of the tunnel.

3D structures of carnitine palmitoyltransferase

Updated on 16-January-2024

Beta oxidation – hCPT I regulatory domain – human - NMR
2rcu - 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

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