Carnitine palmitoyltransferase
From Proteopedia
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| - | '''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 | + | |
| + | == 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.<br /> | ||
| + | * '''CPT I''' is a membrane protein which substitutes the CoA in the long-chain fatty acids by carnitine. After entering the mitochondria.<br /> | ||
| + | * '''CPT II''' exchanges the carnitine by CoA, enabling the oxidation of the long-chain fatty acids. | ||
==Disease == | ==Disease == | ||
| - | CPT I deficiency prevents the body from using certain fats for energy, particularly during fasting. | + | 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== | ==3D structures of carnitine palmitoyltransferase== | ||
Revision as of 09:20, 19 November 2015
Contents |
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
