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.<br /> | '''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 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. | + | * '''CPT II''' exchanges the carnitine by CoA, enabling the oxidation of the long-chain fatty acids.<ref>PMID:15363638</ref> |
==Disease == | ==Disease == |
Revision as of 08:54, 22 November 2015
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References
- ↑ Bonnefont JP, Djouadi F, Prip-Buus C, Gobin S, Munnich A, Bastin J. Carnitine palmitoyltransferases 1 and 2: biochemical, molecular and medical aspects. Mol Aspects Med. 2004 Oct-Dec;25(5-6):495-520. PMID:15363638 doi:http://dx.doi.org/10.1016/j.mam.2004.06.004
- ↑ Rufer AC, Lomize A, Benz J, Chomienne O, Thoma R, Hennig M. Carnitine palmitoyltransferase 2: analysis of membrane association and complex structure with a substrate analog. FEBS Lett. 2007 Jul 10;581(17):3247-52. Epub 2007 Jun 8. PMID:17585909 doi:10.1016/j.febslet.2007.05.080