Carnitine palmitoyltransferase I (CPT1) also known as carnitine acyltransferase I or CAT1 is a mitochondrial enzyme.[1][2] In muscle and other non-liver tissues, CPT1 is associated with the outer mitochondrial membrane. CPT1 mediates the transport of long chain fatty acids across the membrane by binding them to carnitine. This enzyme can be inhibited by malonyl CoA.
Clinical significance
The "CPT1A" form is associated with carnitine palmitoyltransferase I deficiency.[3]
Acyl-CoA from cytosol to the mitochondrial matrix.gif
See also
References
- ^ van der Leij FR, Huijkman NC, Boomsma C, Kuipers JR, Bartelds B (2000). "Genomics of the human carnitine acyltransferase genes". Mol. Genet. Metab. 71 (1-2): 139–53. doi:10.1006/mgme.2000.3055. PMID 11001805.
- ^ Bonnefont JP, Djouadi F, Prip-Buus C, Gobin S, Munnich A, Bastin J (2004). "Carnitine palmitoyltransferases 1 and 2: biochemical, molecular and medical aspects". Mol. Aspects Med. 25 (5-6): 495–520. doi:10.1016/j.mam.2004.06.004. PMID 15363638.
- ^ Ogawa E, Kanazawa M, Yamamoto S, Ohtsuka S, Ogawa A, Ohtake A, Takayanagi M, Kohno Y (2002). "Expression analysis of two mutations in carnitine palmitoyltransferase IA deficiency". J. Hum. Genet. 47 (7): 342–7. doi:10.1007/s100380200047. PMID 12111367.
|
Mitochondrial enzymes and transporters |
|
| Outer membrane |
|
|
| Intermembrane space |
|
|
| Inner membrane |
|
|
| Matrix |
citric acid cycle ( Citrate synthase, Aconitase, Isocitrate dehydrogenase, Oxoglutarate dehydrogenase, Succinyl coenzyme A synthetase, Fumarase, Malate dehydrogenase)
anaplerotic reactions (Aspartate transaminase, Glutamate dehydrogenase, Pyruvate dehydrogenase complex)
urea cycle (Carbamoyl phosphate synthetase I, Ornithine transcarbamylase, N-Acetylglutamate synthase)
alcohol metabolism ( ALDH2)
|
|
| Other/to be sorted |
|
|
| Mitochondrial DNA |
Complex I ( MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND4L, MT-ND5, MT-ND6) - Complex III ( MT-CYB) - Complex IV ( MT-CO1, MT-CO2, MT-CO3)
ATP synthase (MT-ATP6, MT-ATP8)
tRNA ( MT-TA, MT-TC, MT-TD, MT-TE, MT-TF, MT-TG, MT-TH, MT-TI, MT-TK, MT-TL1, MT-TL2, MT-TM, MT-TN, MT-TP, MT-TQ, MT-TR, MT-TS1, MT-TS2, MT-TT, MT-TV, MT-TW, MT-TY)
|
|
This entry is from Wikipedia, the leading user-contributed encyclopedia. It may not have been reviewed by professional editors (see full disclaimer)
