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.

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

CPT I contains an extra ca. 160 amino acids domain at its N terminal.

3D structures of carnitine palmitoyltransferase

Updated on 19-November-2015

2h4t, 2fyo, 2deb – rCPT II – rat

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