Gene Symbol: Cpt2
Description: carnitine palmitoyltransferase 2
Alias: CPTII, carnitine O-palmitoyltransferase 2, mitochondrial, CPT II, carnitine palmitoyltransferase II
Species: rat
Products:     Cpt2

Top Publications

  1. Brown N, Esser V, Gonzalez A, Evans C, Slaughter C, Foster D, et al. Mitochondrial import and processing of rat liver carnitine palmitoyltransferase II defines the amino terminus of the mature protein. Possibility of differential modification of the rat and human isoforms. J Biol Chem. 1991;266:15446-9 pubmed
    ..35S]Methionine-labeled porcine heart citrate synthase (used here as a positive control) and rat liver carnitine palmitoyltransferase II (CPT II) were generated by in vitro transcription and translation of their cDNA constructs in ..
  2. Woeltje K, Esser V, Weis B, Sen A, Cox W, McPhaul M, et al. Cloning, sequencing, and expression of a cDNA encoding rat liver mitochondrial carnitine palmitoyltransferase II. J Biol Chem. 1990;265:10720-5 pubmed
    We report the isolation and characterization of a full-length cDNA encoding rat liver carnitine palmitoyltransferase II (CPT II). Beginning with the purified protein CNBr fragments were generated and sequenced...
  3. Rufer A, Lomize A, Benz J, Chomienne O, Thoma R, Hennig M. Carnitine palmitoyltransferase 2: analysis of membrane association and complex structure with a substrate analog. FEBS Lett. 2007;581:3247-52 pubmed
    ..The protein interacts with the membrane as a functional monomer and the calculations confirm the presence of a membrane association domain that consists of layers of hydrophobic and positively charged residues. ..
  4. Console L, Giangregorio N, Indiveri C, Tonazzi A. Carnitine/acylcarnitine translocase and carnitine palmitoyltransferase 2 form a complex in the inner mitochondrial membrane. Mol Cell Biochem. 2014;394:307-14 pubmed publisher
  5. Zheng G, Dai J, Woldegiorgis G. Identification by mutagenesis of a conserved glutamate (Glu487) residue important for catalytic activity in rat liver carnitine palmitoyltransferase II. J Biol Chem. 2002;277:42219-23 pubmed
    ..carnitine palmitoyltransferase I (CPTI), which is malonyl coA-sensitive and detergent-labile; and carnitine palmitoyltransferase II (CPTII), which is malonyl coA-insensitive and detergent-stable...
  6. Hsiao Y, Jogl G, Esser V, Tong L. Crystal structure of rat carnitine palmitoyltransferase II (CPT-II). Biochem Biophys Res Commun. 2006;346:974-80 pubmed
    b>Carnitine palmitoyltransferase II (CPT-II) has a crucial role in the beta-oxidation of long-chain fatty acids in mitochondria. We report here the crystal structure of rat CPT-II at 1.9A resolution...
  7. Rufer A, Thoma R, Hennig M. Structural insight into function and regulation of carnitine palmitoyltransferase. Cell Mol Life Sci. 2009;66:2489-501 pubmed publisher
    ..However, a number of unresolved questions regarding the biochemistry and pharmacology of CPT enzymes remain and are addressed in this review. ..
  8. Taroni F, Verderio E, Fiorucci S, Cavadini P, Finocchiaro G, Uziel G, et al. Molecular characterization of inherited carnitine palmitoyltransferase II deficiency. Proc Natl Acad Sci U S A. 1992;89:8429-33 pubmed
    Deficiency of carnitine palmitoyltransferase II (CPTase II; palmitoyl-CoA:L-carnitine O-palmitoyltransferase, EC is a clinically heterogeneous autosomal recessive disorder of energy metabolism...
  9. Verderio E, Cavadini P, Montermini L, Wang H, Lamantea E, Finocchiaro G, et al. Carnitine palmitoyltransferase II deficiency: structure of the gene and characterization of two novel disease-causing mutations. Hum Mol Genet. 1995;4:19-29 pubmed
    ..To facilitate the identification of disease-causing mutations in the CPT II gene (CPT1), we have established the genomic organization of this gene...

More Information


  1. de Vries Y, Arvidson D, Waterham H, Cregg J, Woldegiorgis G. Functional characterization of mitochondrial carnitine palmitoyltransferases I and II expressed in the yeast Pichia pastoris. Biochemistry. 1997;36:5285-92 pubmed
    ..the inner mitochondrial membrane by the carnitine/acylcarnitine translocase and converted back to acyl-CoA by CPTII. Although CPTII has been examined in detail, studies on CPTI have been hampered by an inability to purify CPTI in ..
  2. Vladutiu G, Bennett M, Smail D, Wong L, Taggart R, Lindsley H. A variable myopathy associated with heterozygosity for the R503C mutation in the carnitine palmitoyltransferase II gene. Mol Genet Metab. 2000;70:134-41 pubmed
    Adult-onset carnitine palmitoyltransferase II (CPT II) deficiency is an autosomal recessive disease characterized by muscle pain and stiffness with rhabdomyolysis and myoglobinuria in severe cases...
  3. Rufer A, Thoma R, Benz J, Stihle M, Gsell B, De Roo E, et al. The crystal structure of carnitine palmitoyltransferase 2 and implications for diabetes treatment. Structure. 2006;14:713-23 pubmed
    ..Mapping of mutations described for CPT-2 deficiency, a hereditary disorder of lipid metabolism, implies effects on substrate recognition and structural integrity of CPT-2. ..
  4. Gu J, Yao M, Yang J, Cai Y, Zheng W, Wang L, et al. Mitochondrial carnitine palmitoyl transferase-II inactivity aggravates lipid accumulation in rat hepatocarcinogenesis. World J Gastroenterol. 2017;23:256-264 pubmed publisher
    ..05). Low CPT-II expression might lead to abnormal hepatic lipid accumulation, which should promote the malignant transformation of hepatocytes. ..