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Gene Review

Cpt2  -  carnitine palmitoyltransferase 2

Rattus norvegicus

Synonyms: CPT II, Carnitine O-palmitoyltransferase 2, mitochondrial, Carnitine palmitoyltransferase II, Cpt-2
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Disease relevance of Cpt2

  • The cDNA encoding rat liver carnitine palmitoyltransferase II (CPT-II) was heterologously expressed using a recombinant baculovirus/insect cell system [1].
  • Unlike Escherichia coli, the baculovirus-infected insect cells expressed mostly soluble active recombinant CPT-II (rCPT-II) [1].
  • This study was devised to investigate the effect of CARN on altered CPT I and CPT II activity in the cardiomyopathy associated with ADR therapy [2].
  • This study supports the concept that ADR toxicity results from the inhibition of both CPT I and CPT II activities and that one of the causes of ADR-induced cardiomyopathy is a result of globally impaired fatty acid oxidation [2].
  • These changes in metabolic zonation, together with decreased CPT II activity, may contribute to the aggravation of cachexia [3].

High impact information on Cpt2


Chemical compound and disease context of Cpt2


Biological context of Cpt2

  • The predicted amino acid sequence of CPT II shows strong identity with those of two other acyltransferases, namely, rat liver peroxisomal carnitine octanoyltransferase and porcine choline acetyltransferase [10].
  • These data indicate that the catalytic subunit of CPTo/CPT-I is the same as CPTi/CPT-II [7].
  • The overall structure shares strong similarity to those of short- and medium-chain carnitine acyltransferases, although detailed structural differences in the active site region have a significant impact on the substrate selectivity of CPT-II [11].
  • As CPT-1 and CPT-2 are both required for the oxidation of long-chain fatty acids in mitochondria, it can be concluded that inhibition of fatty acid oxidation per se is not responsible for cell growth, but rather the accumulation of a metabolite, probably long-chain acylcoenzyme A [9].

Anatomical context of Cpt2

  • Inhibition of transferase activity by 4-THA and malonyl-CoA was attenuated in mitochondria which had been solubilized with octyl glucoside to expose the latent form of carnitine palmitoyltransferase (CPT-II), suggesting that the inhibition was specific for CPT-I [12].
  • Since carnitine-dependent fatty acid oxidation is modulated by insulin in a variety of tissues, the effects of 1.7 microM insulin on the mitochondrial enzyme(s), carnitine palmitoyltransferase (malonyl-CoA-sensitive CPT-I and the matrix-facing CPT-II), were studied in neonatal rat cardiac myocytes cultured in the absence of serum [13].
  • CPT-I activity in the insulin-supplemented, serum-free cultures is 57% higher (P < 0.002) than CPT-I activity in cells cultured in the absence of insulin; CPT-II activity is also significantly increased (P < 0.01) in the presence of insulin [13].
  • In the cardiac cells treated with 5 microM digitonin, CPT-II contamination of CPT activity is 0.62% as quantitated by citrate synthase activity present in damaged myocytes under assay conditions [14].
  • Our objective was to isolate from rat liver mitochondria the malonyl-CoA-regulated and detergent-labile enzyme, carnitine palmitoyltransferase I (CPT I), whose properties and relationship to CPT II have been the subject of debate [15].

Associations of Cpt2 with chemical compounds


Regulatory relationships of Cpt2

  • The tetradecanamidomethyl analogue of (2R,6S)-1 activated CPT-I but inhibited CPT-II [19].

Other interactions of Cpt2


Analytical, diagnostic and therapeutic context of Cpt2


  1. Over-expression and characterization of active recombinant rat liver carnitine palmitoyltransferase II using baculovirus. Johnson, T.M., Mann, W.R., Dragland, C.J., Anderson, R.C., Nemecek, G.M., Bell, P.A. Biochem. J. (1995) [Pubmed]
  2. Effect of L-carnitine supplementation on cardiac carnitine palmitoyltransferase activities and plasma carnitine concentrations in adriamycin-treated rats. Yoon, H.R., Hong, Y.M., Boriack, R.L., Bennett, M.J. Pediatr. Res. (2003) [Pubmed]
  3. Cancer cachexia modifies the zonal distribution of lipid metabolism-related proteins in rat liver. Kazantzis, M., Seelaender, M.C. Cell Tissue Res. (2005) [Pubmed]
  4. Effects of benfluorex on fatty acid and glucose metabolism in isolated rat hepatocytes: from metabolic fluxes to gene expression. Kohl, C., Ravel, D., Girard, J., Pégorier, J.P. Diabetes (2002) [Pubmed]
  5. Elucidation of the mechanism by which (+)-acylcarnitines inhibit mitochondrial fatty acid transport. Baillet, L., Mullur, R.S., Esser, V., McGarry, J.D. J. Biol. Chem. (2000) [Pubmed]
  6. The malonyl-CoA-sensitive form of carnitine palmitoyltransferase is not localized exclusively in the outer membrane of rat liver mitochondria. Hoppel, C.L., Kerner, J., Turkaly, P., Turkaly, J., Tandler, B. J. Biol. Chem. (1998) [Pubmed]
  7. Characterization of the malonyl-CoA-sensitive carnitine palmitoyltransferase (CPTo) of a rat heart mitochondrial particle. Evidence that the catalytic unit is CPTi. Kerner, J., Zaluzec, E., Gage, D., Bieber, L.L. J. Biol. Chem. (1994) [Pubmed]
  8. Catalytically important domains of rat carnitine palmitoyltransferase II as determined by site-directed mutagenesis and chemical modification. Evidence for a critical histidine residue. Brown, N.F., Anderson, R.C., Caplan, S.L., Foster, D.W., McGarry, J.D. J. Biol. Chem. (1994) [Pubmed]
  9. Comparison of the effects of carnitine palmitoyltransferase-1 and -2 inhibitors on rat heart hypertrophy. Hülsmann, W.C., Peschechera, A., Schneijdenberg, C.T., Verkleij, A.J. Cardioscience. (1994) [Pubmed]
  10. Cloning, sequencing, and expression of a cDNA encoding rat liver mitochondrial carnitine palmitoyltransferase II. Woeltje, K.F., Esser, V., Weis, B.C., Sen, A., Cox, W.F., McPhaul, M.J., Slaughter, C.A., Foster, D.W., McGarry, J.D. J. Biol. Chem. (1990) [Pubmed]
  11. Crystal structure of rat carnitine palmitoyltransferase II (CPT-II). Hsiao, Y.S., Jogl, G., Esser, V., Tong, L. Biochem. Biophys. Res. Commun. (2006) [Pubmed]
  12. Inhibition of hepatic fatty acid oxidation at carnitine palmitoyltransferase I by the peroxisome proliferator 2-hydroxy-3-propyl-4-[6-(tetrazol-5-yl) hexyloxy]acetophenone. Foxworthy, P.S., Eacho, P.I. Biochem. J. (1988) [Pubmed]
  13. Insulin-associated changes in carnitine palmitoyltransferase in cultured neonatal rat cardiac myocytes. Hudson, E.K., Liu, M.H., Buja, L.M., McMillin, J.B. J. Mol. Cell. Cardiol. (1995) [Pubmed]
  14. Kinetic properties of carnitine palmitoyltransferase I in cultured neonatal rat cardiac myocytes. McMillin, J.B., Wang, D., Witters, L.A., Buja, L.M. Arch. Biochem. Biophys. (1994) [Pubmed]
  15. Inhibitors of mitochondrial carnitine palmitoyltransferase I limit the action of proteases on the enzyme. Isolation and partial amino acid analysis of a truncated form of the rat liver isozyme. Esser, V., Kuwajima, M., Britton, C.H., Krishnan, K., Foster, D.W., McGarry, J.D. J. Biol. Chem. (1993) [Pubmed]
  16. Cholate extracts of mitochondrial outer membranes increase inhibition by malonyl-CoA of carnitine palmitoyltransferase-I by a mechanism involving phospholipids. Mynatt, R.L., Greenhaw, J.J., Cook, G.A. Biochem. J. (1994) [Pubmed]
  17. Mitochondrial 3-hydroxy-3-methylglutaryl coenzyme A synthase and carnitine palmitoyltransferase II as potential control sites for ketogenesis during mitochondrion and peroxisome proliferation. Madsen, L., Garras, A., Asins, G., Serra, D., Hegardt, F.G., Berge, R.K. Biochem. Pharmacol. (1999) [Pubmed]
  18. 3-Thia fatty acid treatment, in contrast to eicosapentaenoic acid and starvation, induces gene expression of carnitine palmitoyltransferase-II in rat liver. Madsen, L., Berge, R.K. Lipids (1999) [Pubmed]
  19. Stereoisomeric acylamidomorpholinium carnitine analogues: selective inhibitors of carnitine palmitoyltransferase I and II. Savle, P.S., Pande, S.V., Lee, T.S., Gandour, R.D. Bioorg. Med. Chem. Lett. (1999) [Pubmed]
  20. Proteomic analysis of mitochondrial proteins in cardiomyocytes from chronic stressed rat. Liu, X.H., Qian, L.J., Gong, J.B., Shen, J., Zhang, X.M., Qian, X.H. Proteomics (2004) [Pubmed]
  21. Carnitine palmitoyltransferase II specificity towards beta-oxidation intermediates--evidence for a reverse carnitine cycle in mitochondria. Ventura, F.V., Ijlst, L., Ruiter, J., Ofman, R., Costa, C.G., Jakobs, C., Duran, M., Tavares de Almeida, I., Bieber, L.L., Wanders, R.J. Eur. J. Biochem. (1998) [Pubmed]
  22. Inhibition of carnitine palmitoyltransferase 1 by phenylalkyloxiranecarboxylic acid and its influence on lipolysis and glucose metabolism in isolated, perfused hearts of streptozotocin-diabetic rats. Rösen, P., Reinauer, H. Metab. Clin. Exp. (1984) [Pubmed]
  23. Increased muscle carnitine palmitoyltransferase II mRNA after increased contractile activity. Yan, Z., Salmons, S., Jarvis, J., Booth, F.W. Am. J. Physiol. (1995) [Pubmed]
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