1. McGarry, J. D., Mannaerts, G. P., and Foster, D. W. (1977) A possible
role for malonyl-CoA in the regulation of hepatic fatty acid oxidation
and ketogenesis. J. Clin. Invest. 60, 265–270.
2. McGarry, J. D., Mannaerts, G. P., Foster, and D. W. (1978) Characteristics
of fatty acid oxidation in rat liver homogenates and the inhibitory
effect of malonyl-CoA. Biochim. Biophys. Acta 530, 305–313.
3. McGarry, J. D., and Brown, N. F. (1997) The mitochondrial carnitine
palmitoyltransferase system. From concept to molecular analysis. Eur.
J. Biochem. 244, 1–14.
4. Saggerson, E. D. (1982) Carnitine acyltransferase activities in rat liver
and heart measured with palmitoyl-CoA and octanoyl-CoA—latency,
effects of K1, bivalent metal ions and malonyl-CoA. Biochem. J. 202,
397–405.
5. Park, E. A., and Cook, G. A. (1998) Differential regulation in the heart
of mitochondrial carnitine palmitoyltransferase-I muscle and liver isoforms.
Mol. Cell Biochem. 180, 27–32.
6. Paulson, D. J., Ward, K. M., and Shug, A. L. (1984) Malonyl CoA inhibition
of carnitine palmityltransferase in rat heart mitochondria. FEBS
Lett. 176, 381–384.
7. Price, N., van der Leij, F., Jackson, V., Corstorphine, C., Thomson, R.,
Sorensen, A., and Zammit, V. (2002) A novel brain-expressed protein
related to carnitine palmitoyltransferase I. Genomics 80, 433–442.
8. Wolfgang, M. J., Kurama, T., Dai, Y., Suwa, A., Asaumi, M., Matsumoto,
S., Cha, S. H., Shimokawa, T., and Lane, M. D. (2006) The
brain-specific carnitine palmitoyltransferase-1c regulates energy homeostasis.
Proc. Natl. Acad. Sci USA 103, 7282–7287.
9. Sierra, A. Y., Gratacos, E., Carrasco, P. Clotet, J., Urena, J., Serra, D.,
Asins, G., Hegardt, F. G., and Casals, N. (2008) CPT1c is localized in
endoplasmic reticulum of neurons and has carnitine palmitoyltransferase
activity. J. Biol. Chem, 283, 6878–6885.
10. Dai, Y., Wolfgang, M. J., Cha, S. H., and Lane, M. D. (2007) Localization
and effect of ectopic expression of CPT1c in CNS feeding centers.
Biochem. Biophys. Res. Commun. 359, 469–474.
11. Ontko, J. A., and Johns, M. L. (1980) Evaluation of malonyl-CoA in
the regulation of long-chain fatty acid oxidation in the liver. Evidence
for an unidentified regulatory component of the system. Biochem. J.
192, 959–962.
12. Cook, G. A., Otto, D. A., and Cornell, N. W. (1980) Differential inhibition
of ketogenesis by malonyl-CoA in mitochondria from fed and
starved rats. Biochem. J. 192, 955–958.
13. Robinson, I. N., and Zammit, V. A. (1982) Sensitivity of carnitine acyltransferase
I to malonyl-CoA in isolated rat liver mitochondria is quantitatively
related to hepatic malonyl-CoA concentration. Biochem. J.
206, 177–179.
14. Grantham, B. D. and Zammit, V. A. (1986) Restoration of the properties
of carnitine palmitoyltransferase I in liver mitochondria during refeeding
of starved rats. Biochem. J. 239, 485–488.
15. Grantham, B. D. and Zammit, V. A. (1988) Role of carnitine palmitoyltransferase
I in the regulation of hepatic ketogenesis during the onset
and reversal of chronic diabetes. Biochem. J. 249, 409–414.
16. Moir, A. M. B. and Zammit, V. A. (1995) Acute meal-induced changes
in hepatic glycerolipid metabolism are unimpaired in severely diabetic
rats: implications for the role of insulin. FEBS Lett. 370, 255–258.
17. Moir, A. M. B. and Zammit, V. A. (1992) Selective labelling of hepatic
fatty acids in vivo. Studies on the synthesis and secretion of glycerolipids
in the rat. Biochem. J. 283, 145–149.
18. Moir, A. M. B. and Zammit, V. A. (1993) Rapid switch of hepatic fatty
acid metabolism from oxidation to esterification during diurnal feeding
of meal-fed rats correlates with changes in the properties of acetyl-CoA
carboxylase, but not of carnitine palmitoyl transferase I. Biochem. J.
291, 241–246.
19. Moir, A. M. B., and Zammit, V. A. (1993) Monitoring of changes in hepatic
fatty acid and glycerolipid metabolism during the starved-to-fed transition
in vivo. Studies on awake, unrestrained rats. Biochem. J. 289, 49–55.
20. Brown, N. F., Esser, V., Foster, D. W., and McGarry, J. D. (1994)
Expression of a cDNA for rat liver carnitine palmitoyltransferase I in
yeast establishes that catalytic activity and malonyl-CoA sensitivity reside
in a single polypeptide. J. Biol. Chem. 269, 26438–26642.
21. Zammit, V. A. (1984) Time-dependence of inhibition of carnitine palmitoyltransferase
I by malonyl-CoA in mitochondria isolated from livers
of fed or starved rats. Biochem. J. 218, 379–386.
22. Zammit, V. A., Corstorphine, C. G., Kolodziej, M. P., Fraser, F. (1998)
Lipid molecular order in liver mitochondrial outer membranes, and sensitivity
of carnitine palmitoyltransferase I to malonyl-CoA. Lipids 33,
371–376.
23. Kerner, J. and Bieber, L. (1990) Isolation of a malonyl-CoA-sensitive
CPT/b-oxidation enzyme complex from heart mitochondria. Biochemistry
29, 4326–4334.
24. Fraser, F. and Zammit, V. A. (1998) Enrichment of carnitine palmitoyltransferases
I and II in the contact sites of rat liver mitochondria. Biochem.
J. 329, 225–229.
25. McGarry, J. and Foster, D. W. (1980) Regulation of hepatic fatty acid
oxidation and ketone body production. Annu. Rev. Biochem. 49, 395–
420.
26. Fraser, F., Corstorphine, C. G., and Zammit, V. A. (1997) Topology of
carnitine palmitoyltransferase I in the mitochondrial outer membrane.
Biochem. J. 323, 711–718.
27. van der Leij, F. R., Kram, A. M., Bartelds, B., Roelofsen, H., Smid, G.
B., Takens, J., Zammit, V. A., and Kuipers, J. R. (1999) Cytological
evidence that the C-terminus of carnitine palmitoyltransferase I is on
the cytosolic face of the mitochondrial outer membrane. Biochem. J.
341, 777–784.
28. Zammit, V. (1999) Carnitine acyltransferases: functional significance of
subcellular distribution and membrane topology. Progr. Lipid Res. 38,
199–224.
29. Shi, J., Zhu, H., Arvidson, D. N., and Woldegiorgis, G. (1999) A single
amino acid change (substitution of glutamate 3 with alanine) in the Nterminal
region of rat liver carnitine palmitoyltransferase I abolishes
Malonyl-CoA inhibition and high affinity binding. J. Biol. Chem. 274,
9421–9426.
30. Jackson, V., Price, N., and Zammit, V. (2001) Specificity of the interactions
between Glu-3, Ser-24 and Gln-30 within the N-terminal segment
of rat liver mitochondrial overt carnitine palmitoyltransferase (L-CPT I)
in determining the malonyl-CoA sensitivity of the enzyme. Biochemistry
40, 14629–14634.
31. Jackson, V. N., Cameron, J. M., Fraser, F., Zammit, V. A., and Price,
N. T. (2000) Use of six chimeric proteins to investigate the role of
intramolecular interactions in determining the kinetics of carnitine palmitoyltransferase
I isoforms [in process citation]. J. Biol. Chem. 275,
19560–19566.
32. Jackson, V. N., Zammit, V. A., and Price, N. T. (2000) Identification of
positive and negative determinants of malonyl-CoA- sensitivity and carnitine
affinity within the amino-termini of rat liver- and muscle-type
carnitine palmitoyltransferase I. J. Biol. Chem. 275, 38410–38416.
33. Faye, A., Borthwick, K., Esnous, C., Price, N. T., Gobin, S., Jackson,
V. N., Zammit, V. A., Girard, J., and Prip-Buus, C. (2004) Demonstration
of N/C intramolecular interactions in rat liver carnitine palmitoyltransferase
1 that determine its degree of malonyl-CoA sensitivity.
Biochem. J. 237, 123.
34. Morillas, M., Lopez, V. E., Valencia, A., Serra, D., P.Gomez-Puertas,
Hegardt, F. G., and Asins, G. (2004) Structural model of carnitine palmitoyltransferase
I based on the carnitine acetyltransferase crystal. Biochem.
J. 379, 777–784.
35. Lopez-Vinas, E., Bentebibel, A., Gurunathan, C., Morillas, M., de
Arriaga, D., Serra, D., Asins, G., Hegardt, F. G., and Gomez-Puertas, P.
(2007) Definition by functional and structural analysis of two malonyl-
CoA sites in carnitine palmitoyltransferase 1A. J. Biol. Chem. 282,
18212–18224.
36. Borthwick, K., Jackson, V. N., Price, N. T., and Zammit, V. A. (2006)
The mitochondrial intermembrane loop region of rat carnitine palmitoyltransferase
1A is a major determinant of its malonyl-CoA sensitivity. J.
Biol. Chem. 281, 32946–32952.
37. Curran, A. R. and Engelman, D. M. (2003) Sequence motifs, polar
interactions and conformational changes in helical membrane proteins.
Curr. Opin. Struct. Biol. 13, 412–417.
38. Brandt, J. M., Djouadi, F., and Kelly, D. P. (1998) Fatty acids activate
transcription of the muscle carnitine palmitoyltransferase I gene in cardiac
myocytes via the peroxisome proliferator-activated receptor alpha.
J. Biol. Chem. 273, 23786–23792.
39. Kerner, J., Distler, A. M., Minkler, P., Parland, W., Peterman, S. M.,
and Hoppel, C. L. (2004) Phosphorylation of rat liver mitochondrial carnitine
palmitoyltransferase-I: effect on the kinetic properties of the
enzyme. J. Biol. Chem. 279, 41104–41113.
40. Eaton, S., Fukumoto, K., Stefanutti, G., Spitz, L., Zammit, V. A., and
Pierro, A. (2003) Myocardial carnitine palmitoyltransferase I as a target
for oxidative modification in inflammation and sepsis. Biochem. Soc.
Trans. 31, 1133–1136.
41. Fukumoto, K., Pierro, A., Zammit, V. A., Spitz, L., and Eaton, S.
(2004) Tyrosine nitration of carnitine palmitoyl transferase I during
endotoxaemia in suckling rats. Biochim. Biophys. Acta 1683, 1–6.
42. Leterrier, J. F., Rusakov, B. D., Nelson, B. D., and Linden, M. (1994)
Interactions between brain mitochondria and cytoskeleton: evidence for
specialized outer membrane domains involved in the association of cytoskeleton-
associated proteins to mitochondria in situ and in vitro.
Microsc. Res. Tech. 27, 233–261.
43. Guzman, M., Kolodziej, M. P., Caldwell, A., Corstorphine, C. G., and
Zammit, V. A. (1994) Evidence against direct involvement of phosphorylation
in the activation of carnitine palmitoyltransferase I by akadaic
acid in rat hepatocytes. Biochem. J. 300, 693–699.
44. Guzman, M., Velasco, G., Castro, J., and Zammit, V. A. (1994) Inhibition
of carnitine palmitoyltransferase I by hepatocyte swelling. FEBS
Lett. 344, 239–241.
45. Ruderman, N. and Prentki, M. (2004) AMP kinase and malonyl-CoA:
targets for therapy of the metabolic syndrome. Nat. Rev. Drug Discov.
3, 340–351.
46. Koves, T. R., Ussher, J. R., Noland, R. C., Slentz, D., Mosedale, M.,
Ilkayeva, O., Bain, J., Stevens, R., Dyck, J. R., Newgard, C. B., Lopaschuk,
G. D., and Muoio, D. M. (2008) Mitochondrial overload and
incomplete Fatty Acid oxidation contribute to skeletal muscle insulin
resistance. Cell Metab. 7, 45–56.
47. Dobbins, R. L., Szczepaniak, L. S., Bentley, B., Esser, V., Myhill, J.,
and McGarry, J. D. (2001) Prolonged inhibition of muscle carnitine pal-
mitoyltransferase-1 promotes intramyocellular lipid accumulation and
insulin resistance in rats.[in process citation]. Diabetes 50, 123–130.
48. Prentki, M. and Corkey, B. E. (1996) Are the beta-cell signaling molecules
malonyl-CoA and cystolic long-chain acyl-CoA implicated in multiple
tissue defects of obesity and NIDDM? Diabetes 45, 273–283.
49. Prentki, M., Tornheim, K., and Corkey, B. E. (1997) Signal transduction
mechanisms in nutrient-induced insulin secretion. Diabetologia 40,
S32–S41.
50. Mountjoy, P. D. and Rutter, G. A. (2007) Glucose sensing by hypothalamic
neurones and pancreatic islet cells: AMPle evidence for common
mechanisms? Exp. Physiol. 92, 311–319.
51. Lam, T. K., Pocai, A., R.Gutierrez-Juarez, Obici, S., Bryan, J., Aguilar-
Bryan, L., Schwartz, G. J., and Rossetti, L. (2005) Hypothalamic sensing
of circulating fatty acids is required for glucose homeostasis. Nat. Med.
11, 320–327.
52. Pocai, A., Lam, T. K., Gutierrez-Juarez, R., Obici, S., Schwartz, G. J., Bryan,
J., Aguilar-Bryan, L., and Rossetti, L. (2005) Hypothalamic K(ATP)
channels control hepatic glucose production. Nature 434, 1026–1031.
53. Lam, T. K., Gutierrez-Juarez, R., Pocai, A., Bhanot, S., Tso, P.,
Schwartz, G. J., and Rossetti, L. (2007) Brain glucose metabolism controls
the hepatic secretion of triglyceride-rich lipoproteins. Nat. Med.
13, 171–180.