carnitine o palmitoyltransferase

Summary

Summary: An enzyme that catalyzes reversibly the conversion of palmitoyl-CoA to palmitoylcarnitine in the inner mitochondrial membrane. EC 2.3.1.21.

Top Publications

  1. Bruce C, Brolin C, Turner N, Cleasby M, van der Leij F, Cooney G, et al. Overexpression of carnitine palmitoyltransferase I in skeletal muscle in vivo increases fatty acid oxidation and reduces triacylglycerol esterification. Am J Physiol Endocrinol Metab. 2007;292:E1231-7 pubmed
    ..05). These studies demonstrate that acute overexpression of CPT I in muscle leads to a repartitioning of FAs away from esterification and toward oxidation and highlight the importance of CPT I in regulating muscle FA metabolism. ..
  2. Holloway G, Benton C, Mullen K, Yoshida Y, Snook L, Han X, et al. In obese rat muscle transport of palmitate is increased and is channeled to triacylglycerol storage despite an increase in mitochondrial palmitate oxidation. Am J Physiol Endocrinol Metab. 2009;296:E738-47 pubmed publisher
    ..The additional fatty acid taken up is channeled primarily to esterification, suggesting that upregulation in fatty acid transport as opposed to altered fatty acid oxidation is the major determinant of intramuscular lipid accumulation...
  3. Benton C, Holloway G, Campbell S, Yoshida Y, Tandon N, Glatz J, et al. Rosiglitazone increases fatty acid oxidation and fatty acid translocase (FAT/CD36) but not carnitine palmitoyltransferase I in rat muscle mitochondria. J Physiol. 2008;586:1755-66 pubmed publisher
    ..Moreover, these studies identify for the first time a mechanism by which rosiglitazone stimulates fatty acid oxidation in skeletal muscle, namely the chronic, subcellular relocation of FAT/CD36 to mitochondria...
  4. Campbell S, Tandon N, Woldegiorgis G, Luiken J, Glatz J, Bonen A. A novel function for fatty acid translocase (FAT)/CD36: involvement in long chain fatty acid transfer into the mitochondria. J Biol Chem. 2004;279:36235-41 pubmed
  5. Holloway G, Jain S, Bezaire V, Han X, Glatz J, Luiken J, et al. FAT/CD36-null mice reveal that mitochondrial FAT/CD36 is required to upregulate mitochondrial fatty acid oxidation in contracting muscle. Am J Physiol Regul Integr Comp Physiol. 2009;297:R960-7 pubmed publisher
    ..Nevertheless, the KO animals reveal that FAT/CD36 contributes to the regulation of mitochondrial fatty acid oxidation, which is especially important for meeting the increased metabolic demands during muscle contraction...
  6. Sebastian D, Guitart M, Garcia Martinez C, Mauvezin C, Orellana Gavaldà J, Serra D, et al. Novel role of FATP1 in mitochondrial fatty acid oxidation in skeletal muscle cells. J Lipid Res. 2009;50:1789-99 pubmed publisher
    ..However, etomoxir, an irreversible inhibitor of CPT1, blocked all these effects. These data reveal that FATP1, like FAT/CD36, is associated with mitochondria and has a role in mitochondrial oxidation of fatty acids. ..
  7. Bruce C, Hoy A, Turner N, Watt M, Allen T, Carpenter K, et al. Overexpression of carnitine palmitoyltransferase-1 in skeletal muscle is sufficient to enhance fatty acid oxidation and improve high-fat diet-induced insulin resistance. Diabetes. 2009;58:550-8 pubmed publisher
  8. Bruce C, Thrush A, Mertz V, Bezaire V, Chabowski A, Heigenhauser G, et al. Endurance training in obese humans improves glucose tolerance and mitochondrial fatty acid oxidation and alters muscle lipid content. Am J Physiol Endocrinol Metab. 2006;291:E99-E107 pubmed
  9. Schenk S, Horowitz J. Coimmunoprecipitation of FAT/CD36 and CPT I in skeletal muscle increases proportionally with fat oxidation after endurance exercise training. Am J Physiol Endocrinol Metab. 2006;291:E254-60 pubmed
    ..857, P < 0.003). In conclusion, the findings from this study indicate that exercise training alters the localization of FAT/CD36 and increases its association with CPT I, which may help augment fat oxidation. ..

More Information

Publications94

  1. Sebastian D, Herrero L, Serra D, Asins G, Hegardt F. CPT I overexpression protects L6E9 muscle cells from fatty acid-induced insulin resistance. Am J Physiol Endocrinol Metab. 2007;292:E677-86 pubmed
    ..These results suggest that LCPT I overexpression protects L6E9 myotubes from fatty acid-induced insulin resistance by inhibiting both the accumulation of lipid metabolites and the activation of PKCtheta and PKCzeta. ..
  2. Bezaire V, Bruce C, Heigenhauser G, Tandon N, Glatz J, Luiken J, et al. Identification of fatty acid translocase on human skeletal muscle mitochondrial membranes: essential role in fatty acid oxidation. Am J Physiol Endocrinol Metab. 2006;290:E509-15 pubmed
  3. Miyagawa T, Kawashima M, Nishida N, Ohashi J, Kimura R, Fujimoto A, et al. Variant between CPT1B and CHKB associated with susceptibility to narcolepsy. Nat Genet. 2008;40:1324-8 pubmed publisher
  4. Tunstall R, McAinch A, Hargreaves M, van Loon L, Cameron Smith D. Reduced plasma free fatty acid availability during exercise: effect on gene expression. Eur J Appl Physiol. 2007;99:485-93 pubmed
  5. Puglianiello A, Germani D, Antignani S, Tomba G, Cianfarani S. Changes in the expression of hypothalamic lipid sensing genes in rat model of intrauterine growth retardation (IUGR). Pediatr Res. 2007;61:433-7 pubmed
    ..05) and beta (p=0.005) were significantly decreased. The data presented here support the hypothesis that an abnormal intrauterine milieu can induce changes in hypothalamic lipid sensing. ..
  6. Campbell S, Febbraio M. Effect of ovarian hormones on mitochondrial enzyme activity in the fat oxidation pathway of skeletal muscle. Am J Physiol Endocrinol Metab. 2001;281:E803-8 pubmed
    ..01); however, treatment with E(2) returned beta-HAD activity to C levels. These results suggest that E(2) plays a role in lipid metabolism by increasing the maximal activity of key enzymes in the fat oxidative pathway of skeletal muscle. ..
  7. Simoneau J, Veerkamp J, Turcotte L, Kelley D. Markers of capacity to utilize fatty acids in human skeletal muscle: relation to insulin resistance and obesity and effects of weight loss. FASEB J. 1999;13:2051-60 pubmed
  8. Esser V, Britton C, Weis B, Foster D, McGarry J. Cloning, sequencing, and expression of a cDNA encoding rat liver carnitine palmitoyltransferase I. Direct evidence that a single polypeptide is involved in inhibitor interaction and catalytic function. J Biol Chem. 1993;268:5817-22 pubmed
    ..They also establish unequivocally that CPT I and CPT II are distinct proteins and that inhibitors of CPT I interact within its catalytic domain, not with an associated regulatory component. ..
  9. Price N, van der Leij F, Jackson V, Corstorphine C, Thomson R, Sorensen A, et al. A novel brain-expressed protein related to carnitine palmitoyltransferase I. Genomics. 2002;80:433-42 pubmed
    ..Thus this new CPT I related protein may be specialized for the metabolism of a distinct class of fatty acids involved in brain function. ..
  10. Rasmussen B, Holmbäck U, Volpi E, Morio Liondore B, Paddon Jones D, Wolfe R. Malonyl coenzyme A and the regulation of functional carnitine palmitoyltransferase-1 activity and fat oxidation in human skeletal muscle. J Clin Invest. 2002;110:1687-93 pubmed
    ..07 nmol/g during hyperglycemia with hyperinsulinemia. We conclude that hyperglycemia with hyperinsulinemia increases malonyl-CoA, inhibits functional CPT-1 activity, and shunts LCFA away from oxidation and toward storage in human muscle. ..
  11. van der Leij F, Cox K, Jackson V, Huijkman N, Bartelds B, Kuipers J, et al. Structural and functional genomics of the CPT1B gene for muscle-type carnitine palmitoyltransferase I in mammals. J Biol Chem. 2002;277:26994-7005 pubmed publisher
    ..Therefore, the involvement of splice variation of M-CPT I in the modulation of malonyl-CoA inhibition of fatty acid oxidation may be less relevant than hitherto assumed...
  12. Rubi B, Antinozzi P, Herrero L, Ishihara H, Asins G, Serra D, et al. Adenovirus-mediated overexpression of liver carnitine palmitoyltransferase I in INS1E cells: effects on cell metabolism and insulin secretion. Biochem J. 2002;364:219-26 pubmed
    ..They also indicate that up-regulation of CPT I contributes to the loss of response to high glucose in beta-cells exposed to fatty acids. ..
  13. Ide T. Effect of dietary alpha-linolenic acid on the activity and gene expression of hepatic fatty acid oxidation enzymes. Biofactors. 2000;13:9-14 pubmed
    ..It was suggested that both substrate specificities and alterations in the activities of the enzymes in beta-oxidation pathway play a significant role in the regulation of the serum lipid concentrations in rats fed alpha-18:3. ..
  14. Dobbins R, Szczepaniak L, Bentley B, Esser V, Myhill J, McGarry J. Prolonged inhibition of muscle carnitine palmitoyltransferase-1 promotes intramyocellular lipid accumulation and insulin resistance in rats. Diabetes. 2001;50:123-30 pubmed
    ..In conclusion, either a diet rich in saturated fat or prolonged inhibition of fatty acid oxidation impairs IMGD in rats via a mechanism related to the accumulation of IMCL. ..
  15. Kennedy S, Stanley W, Panchal A, Mazzeo R. Alterations in enzymes involved in fat metabolism after acute and chronic altitude exposure. J Appl Physiol (1985). 2001;90:17-22 pubmed
    ..These data suggest that acclimatization to high altitude selectively decreases key enzymes in fat utilization and oxidation in the heart, liver, and select skeletal muscles. ..
  16. Yamazaki N, Shinohara Y, Shima A, Yamanaka Y, Terada H. Isolation and characterization of cDNA and genomic clones encoding human muscle type carnitine palmitoyltransferase I. Biochim Biophys Acta. 1996;1307:157-61 pubmed
    ..By Northern analysis, we confirmed the dominant expression of this isoform in heart and skeletal muscle. ..
  17. Pilegaard H, Ordway G, Saltin B, Neufer P. Transcriptional regulation of gene expression in human skeletal muscle during recovery from exercise. Am J Physiol Endocrinol Metab. 2000;279:E806-14 pubmed
  18. Totland G, Madsen L, Klementsen B, Vaagenes H, Kryvi H, Frøyland L, et al. Proliferation of mitochondria and gene expression of carnitine palmitoyltransferase and fatty acyl-CoA oxidase in rat skeletal muscle, heart and liver by hypolipidemic fatty acids. Biol Cell. 2000;92:317-29 pubmed
    ..In the group treated with EPA a lower areal fraction of fat droplets was observed, while the DHA group was similar to the control. This indicates that EPA and DHA have different effects on mitochondrial biogenesis. ..
  19. Cabrero A -, Alegret M, Sanchez R, Adzet T, Laguna J, Vazquez M. Bezafibrate reduces mRNA levels of adipocyte markers and increases fatty acid oxidation in primary culture of adipocytes. Diabetes. 2001;50:1883-90 pubmed
    ..These changes may be involved in the reduction in fat depots and in the improvement of insulin sensitivity observed after bezafibrate treatment. ..
  20. Wang D, Harrison W, Buja L, Elder F, McMillin J. Genomic DNA sequence, promoter expression, and chromosomal mapping of rat muscle carnitine palmitoyltransferase I. Genomics. 1998;48:314-23 pubmed
    ..The muscle specificity of the 5' flanking region was verified by comparison of luciferase expression to that of beta-galactosidase in cardiac myocytes and in HepG2 cells. ..
  21. Prip Buus C, Cohen I, Kohl C, Esser V, McGarry J, Girard J. Topological and functional analysis of the rat liver carnitine palmitoyltransferase 1 expressed in Saccharomyces cerevisiae. FEBS Lett. 1998;429:173-8 pubmed
    ..Thus, the expression in S. cerevisiae is a valid model to study the structure-function relationships of L-CPT 1. ..
  22. Saraiva H, Batista R, Alfradique V, Pinto P, Ribeiro L, Oliveira C, et al. l-carnitine supplementation during vitrification or warming of in vivo-produced ovine embryos does not affect embryonic survival rates, but alters CrAT and PRDX1 expression. Theriogenology. 2018;105:150-157 pubmed publisher
    ..In conclusion, further investigations with different concentrations of LC and tools are needed for improvement of the efficiency of these strategies. ..
  23. Lin X, Shim K, Odle J. Carnitine palmitoyltransferase I control of acetogenesis, the major pathway of fatty acid {beta}-oxidation in liver of neonatal swine. Am J Physiol Regul Integr Comp Physiol. 2010;298:R1435-43 pubmed publisher
    ..The results demonstrate that acetate is the primary product of hepatic mitochondrial beta-oxidation in Sus scrofa and that regulation during early development is mediated primarily via kinetic modulation of CPT I...
  24. Nordstoga K, Christophersen B, Ytrehus B, Espenes A, Osmundsen H, Landsverk T, et al. Pancreatitis associated with hyperlipoproteinaemia type I in mink (Mustela vison): earliest detectable changes occur in mitochondria of exocrine cells. J Comp Pathol. 2006;134:320-8 pubmed
    ..It is concluded that pancreatic lesions associated with hyperlipoproteinaemia start in exocrine cells, and are most probably the result of a metabolic disturbance, possibly a toxic effect of an excess of free fatty acids. ..
  25. Peffer P, Lin X, Odle J. Hepatic beta-oxidation and carnitine palmitoyltransferase I in neonatal pigs after dietary treatments of clofibric acid, isoproterenol, and medium-chain triglycerides. Am J Physiol Regul Integr Comp Physiol. 2005;288:R1518-24 pubmed
    ..1). Collectively, these findings indicate that clofibrate effectively induced hepatic CPT activity concomitant with increased fatty acid beta-oxidation...
  26. Skiba Cassy S, Collin A, Chartrin P, Médale F, Simon J, Duclos M, et al. Chicken liver and muscle carnitine palmitoyltransferase 1: nutritional regulation of messengers. Comp Biochem Physiol B Biochem Mol Biol. 2007;147:278-87 pubmed
  27. Brucknerova I, Bzduch V, Behulova D, Ferianec V, Dubovicky M, Ujhazy E, et al. Reversible asphyxial status in a newborn due to neonatal form of carnitine palmitoyltransferase II deficiency. Neuro Endocrinol Lett. 2008;29:627-30 pubmed
  28. Berthiaume M, Laplante M, Festuccia W, Gélinas Y, Poulin S, Lalonde J, et al. Depot-specific modulation of rat intraabdominal adipose tissue lipid metabolism by pharmacological inhibition of 11beta-hydroxysteroid dehydrogenase type 1. Endocrinology. 2007;148:2391-7 pubmed
  29. DeBerardinis R, Lum J, Thompson C. Phosphatidylinositol 3-kinase-dependent modulation of carnitine palmitoyltransferase 1A expression regulates lipid metabolism during hematopoietic cell growth. J Biol Chem. 2006;281:37372-80 pubmed
    ..Together, these results demonstrate that modulation of CPT1A expression by PI3K-dependent signaling is the major mechanism by which cells suppress beta-oxidation during anabolic growth. ..
  30. Greenberg C, Dilling L, Thompson G, Seargeant L, Haworth J, Phillips S, et al. The paradox of the carnitine palmitoyltransferase type Ia P479L variant in Canadian Aboriginal populations. Mol Genet Metab. 2009;96:201-7 pubmed publisher
    ..It is likely that the P479L variant is of ancient origin and presumably its preservation must have conveyed some advantage. ..
  31. Wensaas A, Rustan A, Just M, Berge R, Drevon C, Gaster M. Fatty acid incubation of myotubes from humans with type 2 diabetes leads to enhanced release of beta-oxidation products because of impaired fatty acid oxidation: effects of tetradecylthioacetic acid and eicosapentaenoic acid. Diabetes. 2009;58:527-35 pubmed publisher
  32. Morash A, Le Moine C, McClelland G. Genome duplication events have led to a diversification in the CPT I gene family in fish. Am J Physiol Regul Integr Comp Physiol. 2010;299:R579-89 pubmed publisher
  33. Lin H, Lu J, Laflamme P, Qiao S, Shayegan B, Bryskin I, et al. Inter-related in vitro effects of androgens, fatty acids and oxidative stress in prostate cancer: a mechanistic model supporting prevention strategies. Int J Oncol. 2010;37:761-6 pubmed
    ..These results therefore support the rationale for PC prevention using 5-alpha reductase inhibitors, dietary restrictions or anti-oxidants, each of which has different inhibitory but complementary effects...
  34. Gilad J, Pirogovsky A, Bartal C. Unmasking of carnitine palmitoyltransferase deficiency during an acute exacerbation of asthma complicated by rhabdomyolysis in a soldier. Mil Med. 2004;169:821-3 pubmed
    ..Given the high incidence of asthma, especially among young adults, a high index of suspicion is needed in order that rhabdomyolysis be promptly diagnosed and treated. ..
  35. Naruta E, Egorov A, Omel ianchik C, Buko V. [The influence of panthotenic acid mitochondrial oxidation and oxidative phosphorylation in liver of rats with alimentary obesity]. Vopr Pitan. 2004;73:3-7 pubmed
  36. Briet F, Keith M, Leong Poi H, Kadakia A, Aba Alkhail K, Giliberto J, et al. Triple nutrient supplementation improves survival, infarct size and cardiac function following myocardial infarction in rats. Nutr Metab Cardiovasc Dis. 2008;18:691-9 pubmed publisher
    ..Our results support the potential cardioprotective impact of TNS during myocardial ischemia. In contrast to carnitine supplementation alone, TNS improved survival as well as cardiac function, gene expression and delayed remodeling. ..
  37. Spiegel R, Shaag A, Gutman A, Korman S, Saada A, Elpeleg O, et al. Severe infantile type of carnitine palmitoyltransferase II (CPT II) deficiency due to homozygous R503C mutation. J Inherit Metab Dis. 2007;30:266 pubmed
    ..Our findings indicate that the mutation R503C should be added to the handful of mutations associated with the severe phenotype when present in the homozygous state or combined with another severe mutation. ..
  38. Lau P, Nixon S, Parton R, Muscat G. RORalpha regulates the expression of genes involved in lipid homeostasis in skeletal muscle cells: caveolin-3 and CPT-1 are direct targets of ROR. J Biol Chem. 2004;279:36828-40 pubmed
    ..In conclusion, we speculate that ROR agonists would increase fatty acid catabolism in muscle and suggest selective activators of ROR may have therapeutic utility in the treatment of obesity and atherosclerosis. ..
  39. Downs S, Mosey J, Klinger J. Fatty acid oxidation and meiotic resumption in mouse oocytes. Mol Reprod Dev. 2009;76:844-53 pubmed publisher
    ..These results indicate that activation of AMPK stimulates meiotic resumption in mouse oocytes by eliminating a block to FAO. ..
  40. Boukouvala E, Leaver M, Favre Krey L, Theodoridou M, Krey G. Molecular characterization of a gilthead sea bream (Sparus aurata) muscle tissue cDNA for carnitine palmitoyltransferase 1B (CPT1B). Comp Biochem Physiol B Biochem Mol Biol. 2010;157:189-97 pubmed publisher
    ..Thus fatty acid metabolism by CPT1B and its control by PPARs are similar in fish and mammals, but multiple genes for CPT1A-like proteins in fish also suggest different and more complex pathways of lipid utilisation than in mammals. ..
  41. Gobin S, Thuillier L, Jogl G, Faye A, Tong L, Chi M, et al. Functional and structural basis of carnitine palmitoyltransferase 1A deficiency. J Biol Chem. 2003;278:50428-34 pubmed
    ..This study provides novel insights into the functionality of CPT1A that may contribute to the design of drugs for the treatment of lipid disorders. ..
  42. van Vlies N, Ruiter J, Doolaard M, Wanders R, Vaz F. An improved enzyme assay for carnitine palmitoyl transferase I in fibroblasts using tandem mass spectrometry. Mol Genet Metab. 2007;90:24-9 pubmed
    ..This inhibition is essential for correct calculation of CPTI activity. In fibroblasts of CPTI-deficient patients, CPTI activity was not detectable and this assay can be used for the diagnosis of CPTI-deficiency. ..
  43. Zhou J, Hegsted M, McCutcheon K, Keenan M, Xi X, Raggio A, et al. Peptide YY and proglucagon mRNA expression patterns and regulation in the gut. Obesity (Silver Spring). 2006;14:683-9 pubmed
    ..Our data provide evidence that the distal part of the gut has the ability to sense nutrients such as butyrate, resulting in the up-regulation of PYY and proglucagon gene expression. ..
  44. Bi Y, Cai M, Liang H, Sun W, Li X, Wang C, et al. Increased carnitine palmitoyl transferase 1 expression and decreased sterol regulatory element-binding protein 1c expression are associated with reduced intramuscular triglyceride accumulation after insulin therapy in high-fat-diet and streptozotocin. Metabolism. 2009;58:779-86 pubmed publisher
    ..These results suggested that early insulin reduced intramuscular triglyceride levels in diabetic rats potentially through amelioration of lipid dysfunction and inhibition of lipid synthesis. ..
  45. Minnich A, Tian N, Byan L, Bilder G. A potent PPARalpha agonist stimulates mitochondrial fatty acid beta-oxidation in liver and skeletal muscle. Am J Physiol Endocrinol Metab. 2001;280:E270-9 pubmed
    ..These observations suggest an important mechanism for the biological effects of PPARalpha agonists...
  46. Huang T, Peng G, Kota B, Li G, Yamahara J, Roufogalis B, et al. Pomegranate flower improves cardiac lipid metabolism in a diabetic rat model: role of lowering circulating lipids. Br J Pharmacol. 2005;145:767-74 pubmed
    ..Our findings suggest that PGF extract improves abnormal cardiac lipid metabolism in ZDF rats by activating PPAR-alpha and thereby lowering circulating lipid and inhibiting its cardiac uptake. ..
  47. Gonzalez Hurtado E, Lee J, Choi J, Selen Alpergin E, Collins S, Horton M, et al. Loss of macrophage fatty acid oxidation does not potentiate systemic metabolic dysfunction. Am J Physiol Endocrinol Metab. 2017;312:E381-E393 pubmed publisher
    ..Macrophage fatty acid oxidation likely plays a correlative, rather than causative, role in systemic metabolic dysfunction. ..
  48. Liu H, Zheng G, Zhu H, Woldegiorgis G. Hormonal and nutritional regulation of muscle carnitine palmitoyltransferase I gene expression in vivo. Arch Biochem Biophys. 2007;465:437-42 pubmed
    ..Overall, the M-CPTI promoter was found to be necessary for the tissue-specific hormonal and dietary regulation of the gene expression. ..
  49. Gudbrandsen O, Rodriguez E, Wergedahl H, Mørk S, Reseland J, Skorve J, et al. Trans-10, cis-12-conjugated linoleic acid reduces the hepatic triacylglycerol content and the leptin mRNA level in adipose tissue in obese Zucker fa/fa rats. Br J Nutr. 2009;102:803-15 pubmed publisher
    ..Hence, the present results suggest that trans-10, cis-12-CLA may have some beneficial effects on lipid metabolism and adiposity but possibly reduces insulin sensitivity. ..
  50. He Y, Khan I, Bai X, Odle J, Xi L. Activation of PPAR? by Oral Clofibrate Increases Renal Fatty Acid Oxidation in Developing Pigs. Int J Mol Sci. 2017;18: pubmed publisher
    ..These findings indicate that PPAR? activation stimulates renal fatty acid oxidation but not ketogenesis. ..
  51. Chegary M, te Brinke H, Doolaard M, IJlst L, Wijburg F, Wanders R, et al. Characterization of L-aminocarnitine, an inhibitor of fatty acid oxidation. Mol Genet Metab. 2008;93:403-10 pubmed
    ..Therefore, we conclude that in intact cells L-AC inhibits CPT2. Combined with our observation that l-AC does not activate PPAR, we suggest that L-AC is useful to simulate a FAO defect in cells from different origin. ..
  52. Watt M, Southgate R, Holmes A, Febbraio M. Suppression of plasma free fatty acids upregulates peroxisome proliferator-activated receptor (PPAR) alpha and delta and PPAR coactivator 1alpha in human skeletal muscle, but not lipid regulatory genes. J Mol Endocrinol. 2004;33:533-44 pubmed
    ..Despite these changes in transcription factors/coactivators, the mRNA content of lipid regulatory proteins was generally unaffected by plasma FFA availability. ..
  53. Larosche I, Letteron P, Fromenty B, Vadrot N, Abbey Toby A, Feldmann G, et al. Tamoxifen inhibits topoisomerases, depletes mitochondrial DNA, and triggers steatosis in mouse liver. J Pharmacol Exp Ther. 2007;321:526-35 pubmed
    ..These combined effects could decrease fat removal from the liver, thus causing hepatic steatosis despite a secondary down-regulation of hepatic fatty acid synthase expression. ..
  54. Price E, Guglielmo C. The effect of muscle phospholipid fatty acid composition on exercise performance: a direct test in the migratory white-throated sparrow (Zonotrichia albicollis). Am J Physiol Regul Integr Comp Physiol. 2009;297:R775-82 pubmed publisher
    ..The fatty acid composition of stored triacylglycerol may instead affect exercise via the preferential use of particular fatty acids by muscles. ..
  55. Dyroy E, Wergedahl H, Skorve J, Gudbrandsen O, Songstad J, Berge R. Thia fatty acids with the sulfur atom in even or odd positions have opposite effects on fatty acid catabolism. Lipids. 2006;41:169-77 pubmed
    ..In summary, the position of the sulfur atom in the alkyl chain, especially whether it is placed in the even or odd position, is crucial for the biological effect of the thia FA. ..
  56. Laforet P, Nicolino M, Eymard B. [New approaches for the treatment of metabolic myopathies]. Rev Neurol (Paris). 2007;163:930-5 pubmed
  57. Lavarías S, Pasquevich M, Dreon M, Heras H. Partial characterization of a malonyl-CoA-sensitive carnitine O-palmitoyltransferase I from Macrobrachium borellii (Crustacea: Palaemonidae). Comp Biochem Physiol B Biochem Mol Biol. 2009;152:364-9 pubmed publisher
    ..Although CPT I displayed kinetic differences with insect and vertebrates, prawn showed a high capacity for energy generation through beta-oxidation of long-chain fatty acids...
  58. Musumeci O, Aguennouz M, Comi G, Rodolico C, Autunno M, Bordoni A, et al. Identification of the infant-type R631C mutation in patients with the benign muscular form of CPT2 deficiency. Neuromuscul Disord. 2007;17:960-3 pubmed
    ..These findings indirectly suggest that other modulators may influence clinical severity of CPT2 deficiency. ..
  59. Zhou W, Simpson P, McFadden J, Townsend C, Medghalchi S, Vadlamudi A, et al. Fatty acid synthase inhibition triggers apoptosis during S phase in human cancer cells. Cancer Res. 2003;63:7330-7 pubmed
    ..The continued ability of TOFA to rescue cancer cells from C75 cytotoxicity implies a proapoptotic role for malonyl-CoA independent of CPT-1 that selectively targets cancer cells as they progress into S phase. ..
  60. Wilcken B. Disorders of the carnitine cycle and detection by newborn screening. Ann Acad Med Singapore. 2008;37:71-3 pubmed
    ..All 4 disorders can be detected by newborn screening, with variable sensitivity. Late-onset CPTII probably cannot be detected. Carnitine transporter, CPTI and late-onset CPTII have proven treatment strategies. ..
  61. Morash A, Bureau D, McClelland G. Effects of dietary fatty acid composition on the regulation of carnitine palmitoyltransferase (CPT) I in rainbow trout (Oncorhynchus mykiss). Comp Biochem Physiol B Biochem Mol Biol. 2009;152:85-93 pubmed publisher
    ..Our present data suggest that changes in gene expression of CPT I and PPARs is the main regulatory mechanism controlling CPT I function in fish using our experimental diet. ..
  62. Chandler M, Chavez P, McElfresh T, Huang H, Harmon C, Stanley W. Partial inhibition of fatty acid oxidation increases regional contractile power and efficiency during demand-induced ischemia. Cardiovasc Res. 2003;59:143-51 pubmed
    ..11%) and anterior wall energy efficiency index (91+/-17 vs. 129+/-10%). Partial inhibition of fatty acid oxidation reduced non-oxidative glycolysis and improved regional contractile power and efficiency during demand-induced ischemia. ..
  63. Violante S, IJlst L, van Lenthe H, de Almeida I, Wanders R, Ventura F. Carnitine palmitoyltransferase 2: New insights on the substrate specificity and implications for acylcarnitine profiling. Biochim Biophys Acta. 2010;1802:728-32 pubmed publisher
  64. Sheng L, Qian Z, Shi Y, Yang L, Xi L, Zhao B, et al. Crocetin improves the insulin resistance induced by high-fat diet in rats. Br J Pharmacol. 2008;154:1016-24 pubmed publisher
  65. Pendergast D, Fisher N, Meksawan K, Doubrava M, Vladutiu G. The distribution of white blood cell fat oxidation in health and disease. J Inherit Metab Dis. 2004;27:89-99 pubmed
    ..Ranges of WBC fat oxidation that are abnormally low (<20 nmol/h per 10(9) WBC, normal 20-35) or high (>35 nmol/h per 10(9) WBC) are proposed based on genetic factors evaluated in this study. ..
  66. Wolkowicz P, Grenett H, Belousova M, Urthaler F. Activation of leptin expression by an inhibitor of carnitine palmitoyltransferase-1. Int J Obes Relat Metab Disord. 2004;28:649-51 pubmed
    ..These results support the conclusion that CPT1-linked fatty acid oxidation is a key modulator of leptin expression in fasting rats. ..
  67. Grynberg A. Effectors of fatty acid oxidation reduction: promising new anti-ischaemic agents. Curr Pharm Des. 2005;11:489-509 pubmed
    ..From the mechanistic research published in this field, enough has now been understood to foresee some future possible targets, mainly related to the cardiomyocyte fatty acid metabolism. ..
  68. Brigadeau F, Gele P, Wibaux M, Marquie C, Martin Nizard F, Torpier G, et al. The PPARalpha activator fenofibrate slows down the progression of the left ventricular dysfunction in porcine tachycardia-induced cardiomyopathy. J Cardiovasc Pharmacol. 2007;49:408-15 pubmed
    ..These data suggest that a clinically relevant dose of fenofibrate does not accelerate but slows down heart failure development in the model of pacing-induced heart failure in large mammals. ..
  69. Saggerson D. Malonyl-CoA, a key signaling molecule in mammalian cells. Annu Rev Nutr. 2008;28:253-72 pubmed publisher
  70. Li L, Wu L, Wang C, Liu L, Zhao Y. Adiponectin modulates carnitine palmitoyltransferase-1 through AMPK signaling cascade in rat cardiomyocytes. Regul Pept. 2007;139:72-9 pubmed
    ..When AMPK and/or p38MAPK was inhibited, gAd-enhanced mRNA expression of CPT-1 was partially reduced. In conclusion, our study suggests that the activation of AMPK signaling cascade participates in the promotion effect of gAd on CPT-1. ..
  71. Folmes C, Lopaschuk G. Role of malonyl-CoA in heart disease and the hypothalamic control of obesity. Cardiovasc Res. 2007;73:278-87 pubmed
    ..Targeting enzymes that control malonyl-CoA levels may be an important therapeutic approach to treating heart disease and obesity. ..
  72. Du M, Ahn D. Dietary CLA affects lipid metabolism in broiler chicks. Lipids. 2003;38:505-11 pubmed
    ..CLA treatment did not influence total FFA content in plasma; however, there was a significant difference in the composition of FFA. Dietary CLA reduced the content of linoleic and arachidonic acids in both plasma and liver. ..
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