Reactome | CPT1A transfers PALM to CAR

CPT1A transfers PALM to CAR

Stable Identifier

R-HSA-200406

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol, mitochondrial outer membrane, mitochondrial intermembrane space

Synonyms

palmitoyl-CoA + carnitine => palmitoylcarnitine + CoASH, (R)-carnitine + hexadecanoyl-CoA => CoA + O-hexadecanoyl-(R)-carnitine

ReviewStatus

5/5

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Carnitine palmitoyl transferase 1A (CPT1A) associated with the inner mitochondrial membrane, catalyzes the reaction of palmitoyl-CoA (PALM-CoA) from the cytosol with carnitine (CAR) in the mitochondrial intermembrane space to form palmitoylcarnitine (L-PCARN) and CoA-SH. Three CPT1 isoforms exist; CPT1A, B and C. In the body, CPT1A is most abundant in liver while CPT1B is abundant in muscle. CPT1C is mainly expressed in neurons and localises to the ER and not to the mitochondria. It has little or no enzymatic activity in fatty acid oxidation. Both CPT1A and CPT1B are inhibited by malonyl-CoA (Morillas et al. 2002, 2004; Zammit et al. 2001; Zhu et al. 1997). Mutations in CPT1A are associated with defects in fatty acid metabolism and fasting intolerance, consistent with the role assigned to CPT1 from studies in vitro and in animal models (IJlst et al. 1998; Gobin et al. 2003).
In the nucleus, cellular retinoic acid-binding protein 1 or 2 (CRABP1 or 2), bound to all-trans-retinoic acid (atRA), directly binds to the heterodimeric complex of retinoic acid receptor alpha RXRA) and peroxisome proliferator-activated receptor delta (PPARD). When bound to PPARD, atRA can significantly increase the expression of proteins involved in fatty acid oxidation such as CPT1A via its induction of PPARD (Amengual et al. 2012).

Literature References

PubMed ID	Title	Journal	Year
9691089	Molecular basis of hepatic carnitine palmitoyltransferase I deficiency IJlst, L, Mandel, H, Oostheim, W, Ruiter, JP, Gutman, A, Wanders, RJA	J Clin Invest	1998
14711372	Structural model of carnitine palmitoyltransferase I based on the carnitine acetyltransferase crystal Morillas, M, López-Viñas, E, Valencia, A, Serra, D, Gomez-Puertas, P, Hegardt, FG, Asins, G	Biochem J	2004
9344464	Functional studies of yeast-expressed human heart muscle carnitine palmitoyltransferase I Zhu, H, Shi, J, de Vries, Y, Arvidson, DN, Cregg, JM, Woldegiorgis, G	Arch Biochem Biophys	1997
11790793	Structural model of a malonyl-CoA-binding site of carnitine octanoyltransferase and carnitine palmitoyltransferase I: mutational analysis of a malonyl-CoA affinity domain Morillas, M, Gomez-Puertas, P, Rubi, B, Clotet, J, Arino, J, Valencia, A, Hegardt, FG, Serra, D, Asins, G	J Biol Chem	2002
14517221	Functional and structural basis of carnitine palmitoyltransferase 1A deficiency Gobin, S, Thuillier, L, Jogl, G, Faye, A, Tong, L, Chi, M, Bonnefont, JP, Girard, J, Prip-Buus, C	J Biol Chem	2003
22871568	Induction of carnitine palmitoyl transferase 1 and fatty acid oxidation by retinoic acid in HepG2 cells Amengual, J, Petrov, P, Bonet, ML, Ribot, J, Palou, A	Int. J. Biochem. Cell Biol.	2012
11356169	Structure-function relationships of the liver and muscle isoforms of carnitine palmitoyltransferase I Zammit, VA, Price, NT, Fraser, F, Jackson, VN	Biochem Soc Trans	2001