Gene Symbol: Cpt1b
Description: carnitine palmitoyltransferase 1b, muscle
Alias: Cpt1, Cpt1-m, Cpti, Cpti-m, M-cpti, carnitine O-palmitoyltransferase 1, muscle isoform, muscle-type carnitine palmitoyltransferase I
Species: mouse
Products:     Cpt1b

Top Publications

  1. Ji S, You Y, Kerner J, Hoppel C, Schoeb T, Chick W, et al. Homozygous carnitine palmitoyltransferase 1b (muscle isoform) deficiency is lethal in the mouse. Mol Genet Metab. 2008;93:314-22 pubmed
    ..38% male CPT-1b+/- mice, and 33% female vs. 8% male CPT-1b+/+ mice). Therefore, lethality of homozygous CPT-1b deficiency in the mice is consistent with paucity of human cases. ..
  2. Abbott B, Wood C, Watkins A, Tatum Gibbs K, Das K, Lau C. Effects of perfluorooctanoic acid (PFOA) on expression of peroxisome proliferator-activated receptors (PPAR) and nuclear receptor-regulated genes in fetal and postnatal CD-1 mouse tissues. Reprod Toxicol. 2012;33:491-505 pubmed publisher
    ..The metabolic disruption produced by PFOA may contribute to poor postnatal survival and persistent weight deficits of CD-1 mouse neonates. ..
  3. van der Leij F, Drijfholt A, Kuipers J. Rationale for a conditional knockout mouse model to study carnitine palmitoyltransferase I deficiencies. Adv Exp Med Biol. 1999;466:377-85 pubmed
    ..Of this enzyme, two isoforms are expressed in the neonatal heart: L-CPT1 (the "liver-type" isoform) and M-CPT1 (the "muscle-type" isoform)...
  4. 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
    Transgenic mice carrying the human heart muscle carnitine palmitoyltransferase I (M-CPTI) gene fused to a CAT reporter gene were generated to study the regulation of M-CPTI gene expression...
  5. Zhang Y, Fang X, Dai M, Cao Q, Tan T, He W, et al. Cardiac-specific down-regulation of carnitine palmitoyltransferase-1b (CPT-1b) prevents cardiac remodeling in obese mice. Obesity (Silver Spring). 2016;24:2533-2543 pubmed publisher
    ..CPT-1b represents a potential therapeutic target for the treatment of cardiac dysfunction related to metabolic diseases such as obesity and diabetes. ..
  6. Vandanmagsar B, Warfel J, Wicks S, Ghosh S, Salbaum J, Burk D, et al. Impaired Mitochondrial Fat Oxidation Induces FGF21 in Muscle. Cell Rep. 2016;15:1686-99 pubmed publisher
    ..a mouse model of impaired FAO by deleting carnitine palmitoyltransferase-1b specifically in skeletal muscle (Cpt1b(m-/-)). Cpt1b(m-/-) mice have increased glucose utilization and are resistant to diet-induced obesity...
  7. Haynie K, Vandanmagsar B, Wicks S, Zhang J, Mynatt R. Inhibition of carnitine palymitoyltransferase1b induces cardiac hypertrophy and mortality in mice. Diabetes Obes Metab. 2014;16:757-60 pubmed publisher
    ..To assess the effects of inhibiting the Cpt1b isoform we generated mice with a heart- and skeletal muscle-specific deletion of the Cpt1b, Cpt1b(HM-/-)...
  8. He L, Kim T, Long Q, Liu J, Wang P, Zhou Y, et al. Carnitine palmitoyltransferase-1b deficiency aggravates pressure overload-induced cardiac hypertrophy caused by lipotoxicity. Circulation. 2012;126:1705-16 pubmed publisher
    ..The muscle isoform, CPT1b, is the predominant isoform expressed in the heart...
  9. Dunning K, Cashman K, Russell D, Thompson J, Norman R, Robker R. Beta-oxidation is essential for mouse oocyte developmental competence and early embryo development. Biol Reprod. 2010;83:909-18 pubmed publisher
    ..via beta-oxidation of fatty acids, with the rate-limiting step catalyzed by carnitine palmitoyl transferase I (CPT1B), a process also requiring carnitine...

More Information


  1. Guzmán Ruiz R, Somoza B, Gil Ortega M, Merino B, Cano V, Attane C, et al. Sensitivity of cardiac carnitine palmitoyltransferase to malonyl-CoA is regulated by leptin: similarities with a model of endogenous hyperleptinemia. Endocrinology. 2010;151:1010-8 pubmed publisher
    ..This mechanism appears to be sensitive to both acute and chronic hyperleptinemia. We conclude that this action of leptin is pivotal to drive cardiac metabolism under situations associated to hyperleptinemia. ..
  2. Lai L, Leone T, Zechner C, Schaeffer P, Kelly S, Flanagan D, et al. Transcriptional coactivators PGC-1alpha and PGC-lbeta control overlapping programs required for perinatal maturation of the heart. Genes Dev. 2008;22:1948-61 pubmed publisher
    ..We conclude that PGC-1alpha and PGC-1beta share roles that collectively are necessary for the postnatal metabolic and functional maturation of heart and BAT. ..
  3. Aja S, Landree L, Kleman A, Medghalchi S, Vadlamudi A, McFadden J, et al. Pharmacological stimulation of brain carnitine palmitoyl-transferase-1 decreases food intake and body weight. Am J Physiol Regul Integr Comp Physiol. 2008;294:R352-61 pubmed
    ..These results suggest a role for brain CPT-1 in the regulation of energy balance and implicate CPT-1 stimulation as a pharmacological approach to weight loss. ..
  4. Yamazaki N, Yamanaka Y, Hashimoto Y, Hiramatsu T, Shinohara Y, Terada H. The gene encoding muscle-type carnitine palmitoyltransferase I: comparison of the 5'-upstream region of human and rodent genes. J Biochem. 2003;133:523-32 pubmed
    Muscle-type carnitine palmitoyltransferase I (M-CPTI) is the key enzyme for fatty acid beta-oxidation in heart and skeletal muscles and in adipose tissue...
  5. 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...
  6. 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
  7. Aoyama C, Yamazaki N, Terada H, Ishidate K. Structure and characterization of the genes for murine choline/ethanolamine kinase isozymes alpha and beta. J Lipid Res. 2000;41:452-64 pubmed
    ..The results demonstrated that, while both CK/EK-alpha and -beta genes were composed of 11 major exons, the size of their genes was quite different: 40 kb for CK/EK-alpha, whereas it was only 3.5 kb for CK/EK-beta. ..
  8. Richmond S, Carper M, Lei X, Zhang S, Yarasheski K, Ramanadham S. HIV-protease inhibitors suppress skeletal muscle fatty acid oxidation by reducing CD36 and CPT1 fatty acid transporters. Biochim Biophys Acta. 2010;1801:559-66 pubmed publisher
    ..found no effect of the HIV-PIs on FATP1, FATP4, or FABPpm but both CD36/FAT and carnitine palmitoyltransferase 1 (CPT1) were reduced by all three regimens though ATV/r caused only a small decrease in CPT1, relative to LPV/r or DRV/r...
  9. 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. ..
  10. 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
    ..When the JVS mice were treated with carnitine, CPT I gene expression was repressed to the level of normal mice. Therefore, the increased expression of the CPT I gene was associated with carnitine deficiency. ..
  11. 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. ..
  12. Wicks S, Vandanmagsar B, Haynie K, Fuller S, Warfel J, Stephens J, et al. Impaired mitochondrial fat oxidation induces adaptive remodeling of muscle metabolism. Proc Natl Acad Sci U S A. 2015;112:E3300-9 pubmed publisher
    ..At the whole-body level, these adaptations result in resistance to obesity. ..
  13. 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
    ..Inhibition of CPT I by etomoxir enhanced palmitate-induced cell death and led to a further increase in ceramide synthesis. ..
  14. Brandt J, Djouadi F, Kelly D. Fatty acids activate transcription of the muscle carnitine palmitoyltransferase I gene in cardiac myocytes via the peroxisome proliferator-activated receptor alpha. J Biol Chem. 1998;273:23786-92 pubmed
  15. 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. ..
  16. Lehman J, Barger P, Kovacs A, Saffitz J, Medeiros D, Kelly D. Peroxisome proliferator-activated receptor gamma coactivator-1 promotes cardiac mitochondrial biogenesis. J Clin Invest. 2000;106:847-56 pubmed
    ..These results identify PGC-1 as a critical regulatory molecule in the control of cardiac mitochondrial number and function in response to energy demands. ..
  17. 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. ..
  18. Chick W, Mentzer S, Carpenter D, Rinchik E, Johnson D, You Y. X-ray-induced deletion complexes in embryonic stem cells on mouse chromosome 15. Mamm Genome. 2005;16:661-71 pubmed
    ..Deletions induced in ES cells by X rays vary in size and location of breakpoints, which makes them desirable for mapping and for functional genomics studies. ..
  19. Gentile L, Monti M, Sebastiano V, Merico V, Nicolai R, Calvani M, et al. Single-cell quantitative RT-PCR analysis of Cpt1b and Cpt2 gene expression in mouse antral oocytes and in preimplantation embryos. Cytogenet Genome Res. 2004;105:215-21 pubmed
    ..We have carried out a study to determine the relative number of transcripts of Cpt1b and Cpt2 genes encoding for m-CPT-I and CPT-II enzymes, during mouse preimplantation development...