Gene Symbol: Cpt1a
Description: carnitine palmitoyltransferase 1a, liver
Alias: C730027G07, CPTI, Cpt1, carnitine O-palmitoyltransferase 1, liver isoform, CPT I, CPT1-L, CPTI-L, L-CPT I, carnitine O-palmitoyltransferase I, liver isoform, carnitine palmitoyltransferase 1, liver
Species: mouse
Products:     Cpt1a

Top Publications

  1. Vavrova E, Lenoir V, Alves Guerra M, Denis R, Castel J, Esnous C, et al. Muscle expression of a malonyl-CoA-insensitive carnitine palmitoyltransferase-1 protects mice against high-fat/high-sucrose diet-induced insulin resistance. Am J Physiol Endocrinol Metab. 2016;311:E649-60 pubmed publisher
    ..Carnitine palmitoyltransferase-1 (CPT1) is a key regulatory enzyme of mFAO whose activity is inhibited by malonyl-CoA, a lipogenic intermediate...
  2. Wong B, Wang X, Zecchin A, Thienpont B, Cornelissen I, Kalucka J, et al. The role of fatty acid ?-oxidation in lymphangiogenesis. Nature. 2017;542:49-54 pubmed publisher
    ..Here we report that in transgenic mouse models, LEC-specific loss of CPT1A, a rate-controlling enzyme in fatty acid ?-oxidation, impairs lymphatic development...
  3. Jiang F, Zhang Z, Zhang Y, Wu J, Yu L, Liu S. L-carnitine ameliorates the liver inflammatory response by regulating carnitine palmitoyltransferase I-dependent PPARγ signaling. Mol Med Rep. 2016;13:1320-8 pubmed publisher
    ..The aim of the present study was to examine the role of the CPT I-dependent peroxisome proliferator-activated receptor (PPAR)γ signaling pathway in the ameliorative effect of L-..
  4. Hénique C, Mansouri A, Vavrova E, Lenoir V, Ferry A, Esnous C, et al. Increasing mitochondrial muscle fatty acid oxidation induces skeletal muscle remodeling toward an oxidative phenotype. FASEB J. 2015;29:2473-83 pubmed publisher
    ..mFAO is the main muscle energy source during endurance exercise, with carnitine palmitoyltransferase 1 (CPT1) being the key regulatory enzyme...
  5. Morillas M, Clotet J, Rubi B, Serra D, Arino J, Hegardt F, et al. Inhibition by etomoxir of rat liver carnitine octanoyltransferase is produced through the co-ordinate interaction with two histidine residues. Biochem J. 2000;351 Pt 2:495-502 pubmed
    ..Our data demonstrate that both etomoxir and malonyl-CoA inhibit COT by interacting with the same sites. ..
  6. Treber M, Dai J, Woldegiorgis G. Identification by mutagenesis of conserved arginine and glutamate residues in the C-terminal domain of rat liver carnitine palmitoyltransferase I that are important for catalytic activity and malonyl-CoA sensitivity. J Biol Chem. 2003;278:11145-9 pubmed
    Carnitine palmitoyltransferase I (CPTI) catalyzes the conversion of long chain fatty acyl-CoAs to acylcarnitines in the presence of l-carnitine...
  7. Rao J, Warren G, Estolt Povedano S, Zammit V, Ulmer T. An environment-dependent structural switch underlies the regulation of carnitine palmitoyltransferase 1A. J Biol Chem. 2011;286:42545-54 pubmed publisher
    ..Here, we show that the N-terminal regulatory domain (N) of CPT1A can adopt two complex amphiphilic structural states, termed N? and N?, that interchange in a switch-like manner in ..
  8. Aoi W, Naito Y, Takanami Y, Ishii T, Kawai Y, Akagiri S, et al. Astaxanthin improves muscle lipid metabolism in exercise via inhibitory effect of oxidative CPT I modification. Biochem Biophys Res Commun. 2008;366:892-7 pubmed
    ..Colocalization of fatty acid translocase with carnitine palmitoyltransferase I (CPT I) in skeletal muscle was increased by astaxanthin...
  9. Iliopoulos D, Drosatos K, Hiyama Y, Goldberg I, Zannis V. MicroRNA-370 controls the expression of microRNA-122 and Cpt1alpha and affects lipid metabolism. J Lipid Res. 2010;51:1513-23 pubmed publisher

More Information


  1. Ferrell J, Boehme S, Li F, Chiang J. Cholesterol 7?-hydroxylase-deficient mice are protected from high-fat/high-cholesterol diet-induced metabolic disorders. J Lipid Res. 2016;57:1144-54 pubmed publisher
  2. Miotto P, Steinberg G, Holloway G. Controlling skeletal muscle CPT-I malonyl-CoA sensitivity: the importance of AMPK-independent regulation of intermediate filaments during exercise. Biochem J. 2017;474:557-569 pubmed publisher
    ..Moreover, M-CoA sensitivity appears to be regulated by intermediate filament interaction with CPT-I, a process that is important when metabolic homeostasis is challenged. ..
  3. Tyra H, Spitz D, Rutkowski D. Inhibition of fatty acid oxidation enhances oxidative protein folding and protects hepatocytes from endoplasmic reticulum stress. Mol Biol Cell. 2012;23:811-9 pubmed publisher
    ..Our results suggest that ER stress can be ameliorated through alteration of the oxidizing environment within the ER lumen, and they provide a potential logic for the transient regulation of metabolic pathways by the UPR during stress. ..
  4. Banks A, Kim Muller J, Mastracci T, Kofler N, Qiang L, Haeusler R, et al. Dissociation of the glucose and lipid regulatory functions of FoxO1 by targeted knockin of acetylation-defective alleles in mice. Cell Metab. 2011;14:587-97 pubmed publisher
    ..Thus, acetylation inactivates FoxO1 during nutrient excess whereas deacetylation selectively potentiates FoxO1 activity, protecting against excessive catabolism during nutrient deprivation. ..
  5. Herrero L, Rubi B, Sebastian D, Serra D, Asins G, Maechler P, et al. Alteration of the malonyl-CoA/carnitine palmitoyltransferase I interaction in the beta-cell impairs glucose-induced insulin secretion. Diabetes. 2005;54:462-71 pubmed
    ..The results provide direct support for the hypothesis that the malonyl-CoA/CPTI interaction is a component of a metabolic signaling network that controls insulin secretion.
  6. Nyman L, Cox K, Hoppel C, Kerner J, Barnoski B, Hamm D, et al. Homozygous carnitine palmitoyltransferase 1a (liver isoform) deficiency is lethal in the mouse. Mol Genet Metab. 2005;86:179-87 pubmed
    ..Although the homozygous mutants were not viable, we did find some aspects of haploinsufficiency in the CPT-1a +/- mutants, which will make them an important mouse model for studying the role of CPT-1a in human disease. ..
  7. Warfel J, Vandanmagsar B, Dubuisson O, Hodgeson S, Elks C, Ravussin E, et al. Examination of carnitine palmitoyltransferase 1 abundance in white adipose tissue: implications in obesity research. Am J Physiol Regul Integr Comp Physiol. 2017;312:R816-R820 pubmed publisher
    ..i>Cpt1a is the predominate isoform in adipose tissue from all three species...
  8. 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
    ..Akt exert these effects by suppressing expression of the beta-oxidation enzyme carnitine palmitoyltransferase 1A (CPT1A)...
  9. Abu Elheiga L, Oh W, Kordari P, Wakil S. Acetyl-CoA carboxylase 2 mutant mice are protected against obesity and diabetes induced by high-fat/high-carbohydrate diets. Proc Natl Acad Sci U S A. 2003;100:10207-12 pubmed
    ..These results suggest that ACC2 plays an essential role in controlling fatty acid oxidation and is a potential target in therapy against obesity and related diseases. ..
  10. Louet J, Hayhurst G, Gonzalez F, Girard J, Decaux J. The coactivator PGC-1 is involved in the regulation of the liver carnitine palmitoyltransferase I gene expression by cAMP in combination with HNF4 alpha and cAMP-response element-binding protein (CREB). J Biol Chem. 2002;277:37991-8000 pubmed
    Liver carnitine palmitoyltransferase I catalyzes the transfer of long-chain fatty acids into mitochondria. L-CPT I is considered the rate-controlling enzyme in fatty acid oxidation...
  11. Zhang L, Tian F, Gao X, Wang X, Wu C, Li N, et al. N-3 Polyunsaturated Fatty Acids Improve Liver Lipid Oxidation-Related Enzyme Levels and Increased the Peroxisome Proliferator-Activated Receptor ? Expression Level in Mice Subjected to Hemorrhagic Shock/Resuscitation. Nutrients. 2016;8:237 pubmed publisher
    ..This function is possibly accomplished through activating the peroxisome proliferator-activated receptor-? pathway. ..
  12. Paumen M, Ishida Y, Han H, Muramatsu M, Eguchi Y, Tsujimoto Y, et al. Direct interaction of the mitochondrial membrane protein carnitine palmitoyltransferase I with Bcl-2. Biochem Biophys Res Commun. 1997;231:523-5 pubmed
    To demonstrate interaction between the mitochondrial membrane proteins CPT I and Bcl-2 the yeast two-hybrid system was used. Full-length CPT I was required for binding to Bcl-2...
  13. Schoors S, Bruning U, Missiaen R, Queiroz K, Borgers G, Elia I, et al. Fatty acid carbon is essential for dNTP synthesis in endothelial cells. Nature. 2015;520:192-197 pubmed publisher
    ..Here we report that endothelial loss of CPT1A, a rate-limiting enzyme of fatty acid oxidation (FAO), causes vascular sprouting defects due to impaired ..
  14. Toral M, Romero M, Jiménez R, Mahmoud A, Barroso E, Gómez Guzmán M, et al. Carnitine palmitoyltransferase-1 up-regulation by PPAR-β/δ prevents lipid-induced endothelial dysfunction. Clin Sci (Lond). 2015;129:823-37 pubmed publisher
    ..In conclusion, PPARβ/δ activation restored the lipid-induced endothelial dysfunction by up-regulation of CPT-1, thus reducing DAG accumulation and the subsequent PKC-mediated ROS production and eNOS inhibition. ..
  15. Nyman L, Tian L, Hamm D, Schoeb T, Gower B, Nagy T, et al. Long term effects of high fat or high carbohydrate diets on glucose tolerance in mice with heterozygous carnitine palmitoyltransferase-1a (CPT-1a) deficiency: Diet influences on CPT1a deficient mice. Nutr Diabetes. 2011;1:e14 pubmed
    ..Furthermore, CPT-1a deficient mice had distinct phenotypes dependent on the diet fed demonstrating that both diet and genetics collectively play a role in the development of impaired glucose tolerance. ..
  16. Britton C, Schultz R, Zhang B, Esser V, Foster D, McGarry J. Human liver mitochondrial carnitine palmitoyltransferase I: characterization of its cDNA and chromosomal localization and partial analysis of the gene. Proc Natl Acad Sci U S A. 1995;92:1984-8 pubmed
    Using the cDNA for rat liver mitochondrial carnitine palmitoyltransferase I (CPT I; EC as a probe, we isolated its counterpart as three overlapping clones from a human liver cDNA library...
  17. Jiang F, Zhang Z, Zhang Y, Pan X, Yu L, Liu S. L-Carnitine Ameliorates Cancer Cachexia in Mice Partly via the Carnitine Palmitoyltransferase-Associated PPAR-γ Signaling Pathway. Oncol Res Treat. 2015;38:511-6 pubmed publisher
    ..These ameliorative effects of L-carnitine were lessened by the carnitine palmitoyltransferase I (CPT I) inhibitor, etomoxir...
  18. Britton C, Mackey D, Esser V, Foster D, Burns D, Yarnall D, et al. Fine chromosome mapping of the genes for human liver and muscle carnitine palmitoyltransferase I (CPT1A and CPT1B). Genomics. 1997;40:209-11 pubmed
  19. Zhang Y, Wang C, Tian Y, Zhang F, Xu W, Li X, et al. Inhibition of Poly(ADP-Ribose) Polymerase-1 Protects Chronic Alcoholic Liver Injury. Am J Pathol. 2016;186:3117-3130 pubmed publisher
  20. Jackson Hayes L, Song S, Lavrentyev E, Jansen M, Hillgartner F, Tian L, et al. A thyroid hormone response unit formed between the promoter and first intron of the carnitine palmitoyltransferase-Ialpha gene mediates the liver-specific induction by thyroid hormone. J Biol Chem. 2003;278:7964-72 pubmed
    ..Our data indicate that the T3 stimulates CPT-Ialpha gene expression in the liver through a T3 response unit consisting of the TRE in the promoter and additional factors, C/EBP and USF, bound in the first intron. ..
  21. Takashima S, Ikejima K, Arai K, Yokokawa J, Kon K, Yamashina S, et al. Glycine prevents metabolic steatohepatitis in diabetic KK-Ay mice through modulation of hepatic innate immunity. Am J Physiol Gastrointest Liver Physiol. 2016;311:G1105-G1113 pubmed publisher
    ..It is proposed that glycine is a promising immunonutrient for prevention and treatment of metabolic syndrome-related nonalcoholic steatohepatitis. ..
  22. Palladino E, Wang W, Albert C, Langhi C, Baldán A, Ford D. Peroxisome proliferator-activated receptor-α accelerates α-chlorofatty acid catabolism. J Lipid Res. 2017;58:317-324 pubmed publisher
    ..increases PPAR-α, PPAR-δ, and PPAR-γ activities, as well as mRNA expression of the PPAR-α target genes, CD36, CPT1a, Cyp4a10, and CIDEC...
  23. Knobloch M, Pilz G, Ghesquière B, Kovacs W, Wegleiter T, Moore D, et al. A Fatty Acid Oxidation-Dependent Metabolic Shift Regulates Adult Neural Stem Cell Activity. Cell Rep. 2017;20:2144-2155 pubmed publisher
    ..Quiescent NSPCs show high levels of carnitine palmitoyltransferase 1a (Cpt1a)-dependent FAO, which is downregulated in proliferating NSPCs...
  24. Fonseca T, Fernandes G, McAninch E, Bocco B, Abdalla S, Ribeiro M, et al. Perinatal deiodinase 2 expression in hepatocytes defines epigenetic susceptibility to liver steatosis and obesity. Proc Natl Acad Sci U S A. 2015;112:14018-23 pubmed publisher
    ..The resulting phenotype of the adult ALB-D2KO mouse is dramatic, with greatly reduced susceptibility to diet-induced steatosis, hypertriglyceridemia, and obesity. ..
  25. Yuan H, Niu Y, Liu X, Fu L. Exercise increases the binding of MEF2A to the Cpt1b promoter in mouse skeletal muscle. Acta Physiol (Oxf). 2014;212:283-92 pubmed publisher
    ..Exercise training decreased the abundance of HDAC5 in the nucleus while increasing its abundance in the cytoplasm. Our results indicated that exercise-induced CPT1b expression was at least in part mediated by HDAC5/MEF2A interaction. ..
  26. Kirpich I, Zhang J, Gobejishvili L, Kharebava G, Barker D, Ghare S, et al. Binge ethanol-induced HDAC3 down-regulates Cpt1? expression leading to hepatic steatosis and injury. Alcohol Clin Exp Res. 2013;37:1920-9 pubmed publisher
    ..Hepatic steatosis, injury, expression of HDAC3 and carnitine palmitoyltransferase 1? (CPT1?) were evaluated...
  27. Cox G, Mahaffey C, Frankel W. Identification of the mouse neuromuscular degeneration gene and mapping of a second site suppressor allele. Neuron. 1998;21:1327-37 pubmed
    ..The identification of the nmd gene and mapping of a major suppressor provide new opportunities for understanding mechanisms of motor neuron degeneration. ..
  28. Paumen M, Ishida Y, Muramatsu M, Yamamoto M, Honjo T. Inhibition of carnitine palmitoyltransferase I augments sphingolipid synthesis and palmitate-induced apoptosis. J Biol Chem. 1997;272:3324-9 pubmed
    ..hybridization approach and isolated a cDNA encoding a mouse homolog of carnitine palmitoyltransferase I (CPT I), an enzyme that resides at the outer mitochondrial membrane and facilitates passage of long-chain fatty acids ..
  29. Uenaka R, Kuwajima M, Ono A, Matsuzawa Y, Hayakawa J, Inohara N, et al. Increased expression of carnitine palmitoyltransferase I gene is repressed by administering L-carnitine in the hearts of carnitine-deficient juvenile visceral steatosis mice. J Biochem. 1996;119:533-40 pubmed
    ..Carnitine palmitoyltransferase I (CPT I) was one of the isolated genes...
  30. Gelb B. Genomic structure of and a cardiac promoter for the mouse carnitine palmitoyltransferase II gene. Genomics. 1993;18:651-5 pubmed
    ..This new promoter is the major driver of carnitine palmitoyltransferase II gene expression in heart and may also be active in liver. ..