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20-September-2008 09:55:58 - II Carnitine palmitoyltransferase II Identifiers Symbols CPT2; CPT1; CPTASE External IDs OMIM: 600650 MGI: 109176 HomoloGene: 77 Gene ontology Molecular function: carnitine O-palmitoyltransferase activity acyltransferase activity transferase activity Cellular component: mitochondrion mitochondrial inner membrane membrane Biological process: lipid metabolic process fatty acid metabolic process fatty acid beta-oxidation transport RNA expression pattern More reference expression data Orthologs Human Mouse Entrez 1376 12896 Ensembl ENSG00000157184 ENSMUSG00000028607 Uniprot P23786 Q3TFS0 Refseq NM_000098 mRNA NP_000089 protein NM_009949 mRNA NP_034079 protein Location Chr 1: 53.43 - 53.63 Mb Chr 4: 107.4 - 107.42 Mb Pubmed search 1 2 Carnitine palmitoyltransferase II, also known as CPT2, is a human gene.1 Carnitine palmitoyltransferase II precursor CPT2 is a nuclear protein which is transported to the mitochondrial inner membrane. CPT2 together with carnitine palmitoyltransferase I oxidizes long-chain fatty acids in the mitochondria. Defects in this gene are associated with mitochondrial long-chain fatty-acid LCFA oxidation disorders and carnitine palmitoyltransferase II deficiency.1 Acyl-CoA from cytosol to the mitochondrial matrix.gif Acyl-CoA from cytosol to the mitochondrial matrix.gif References ^ a b Entrez Gene: CPT2 carnitine palmitoyltransferase II. Further reading Bonnefont JP, Demaugre F, Prip-Buus C, et al. 2000. Carnitine palmitoyltransferase deficiencies.. Mol. Genet. Metab. 68 4: 424-40. doi:10.1006/mgme.1999.2938. PMID 10607472. van der Leij FR, Huijkman NC, Boomsma C, et al. 2000. Genomics of the human carnitine acyltransferase genes.. Mol. Genet. Metab. 71 1-2: 139-53. doi:10.1006/mgme.2000.3055. PMID 11001805. Sigauke E, Rakheja D, Kitson K, Bennett MJ 2003. Carnitine palmitoyltransferase II deficiency: a clinical, biochemical, and molecular review.. Lab. Invest. 83 11: 1543-54. PMID 14615409. Minoletti F, Colombo I, Martin AL, et al. 1992. Localization of the human gene for carnitine palmitoyltransferase to 1p13-p11 by nonradioactive in situ hybridization.. Genomics 13 4: 1372-4. PMID 1339389. Taroni F, Verderio E, Fiorucci S, et al. 1992. Molecular characterization of inherited carnitine palmitoyltransferase II deficiency.. Proc. Natl. Acad. Sci. U.S.A. 89 18: 8429-33. PMID 1528846. Finocchiaro G, Taroni F, Rocchi M, et al. 1992. cDNA cloning, sequence analysis, and chromosomal localization of human carnitine palmitoyltransferase.. Proc. Natl. Acad. Sci. U.S.A. 88 23: 10981. PMID 1961767. Finocchiaro G, Taroni F, Rocchi M, et al. 1991. cDNA cloning, sequence analysis, and chromosomal localization of the gene for human carnitine palmitoyltransferase.. Proc. Natl. Acad. Sci. U.S.A. 88 2: 661-5. PMID 1988962. Finocchiaro G, Colombo I, DiDonato S 1991. Purification, characterization and partial amino acid sequences of carnitine palmitoyl-transferase from human liver.. FEBS Lett. 274 1-2: 163-6. PMID 2174799. Verderio E, Cavadini P, Montermini L, et al. 1995. Carnitine palmitoyltransferase II deficiency: structure of the gene and characterization of two novel disease-causing mutations.. Hum. Mol. Genet. 4 1: 19-29. PMID 7711730. Britton CH, Schultz RA, Zhang B, et al. 1995. 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. 92 6: 1984-8. PMID 7892212. Gellera C, Verderio E, Floridia G, et al. 1995. Assignment of the human carnitine palmitoyltransferase II gene CPT1 to chromosome 1p32.. Genomics 24 1: 195-7. doi:10.1006/geno.1994.1605. PMID 7896283. Montermini L, Wang H, Verderio E, et al. 1994. Identification of 5' regulatory regions of the human carnitine palmitoyltransferase II gene.. Biochim. Biophys. Acta 1219 1: 237-40. PMID 8086471. Maruyama K, Sugano S 1994. Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides.. Gene 138 1-2: 171-4. PMID 8125298. Taroni F, Verderio E, Dworzak F, et al. 1993. Identification of a common mutation in the carnitine palmitoyltransferase II gene in familial recurrent myoglobinuria patients.. Nat. Genet. 4 3: 314-20. doi:10.1038/ng0793-314. PMID 8358442. Verderio E, Cavadini P, Pandolfo M, et al. 1993. Two novel sequence polymorphisms of the human carnitine palmitoyltransferase II CPT1 gene.. Hum. Mol. Genet. 2 3: 334. PMID 8499929. Bonnefont JP, Taroni F, Cavadini P, et al. 1996. Molecular analysis of carnitine palmitoyltransferase II deficiency with hepatocardiomuscular expression.. Am. J. Hum. Genet. 58 5: 971-8. PMID 8651281. Suzuki Y, Yoshitomo-Nakagawa K, Maruyama K, et al. 1997. Construction and characterization of a full length-enriched and a 5'-end-enriched cDNA library.. Gene 200 1-2: 149-56. PMID 9373149. Wataya K, Akanuma J, Cavadini P, et al. 1998. Two CPT2 mutations in three Japanese patients with carnitine palmitoyltransferase II deficiency: functional analysis and association with polymorphic haplotypes and two clinical phenotypes.. Hum. Mutat. 11 5: 377-86. doi:10.1002/SICI1098-1004199811:5377::AID-HUMU53.0.CO;2-E. PMID 9600456. Yang BZ, Ding JH, Dewese T, et al. 1998. Identification of four novel mutations in patients with carnitine palmitoyltransferase II CPT II deficiency.. Mol. Genet. Metab. 64 4: 229-36. doi:10.1006/mgme.1998.2711. PMID 9758712. Taggart RT, Smail D, Apolito C, Vladutiu GD 1999. Novel mutations associated with carnitine palmitoyltransferase II deficiency.. Hum. Mutat. 13 3: 210-20. doi:10.1002/SICI1098-1004199913:3210::AID-HUMU53.0.CO;2-0. PMID 10090476. This article on a gene on chromosome 1 is a stub. v d e Transferases: acyltransferases EC 2.3 2.3.1: other than amino-acyl groups mostly acetyltransferases N-Acetylglutamate synthase - Choline acetyltransferase - Acetyl-Coenzyme A acetyltransferase - Dihydrolipoyl transacetylase - Acetyl-CoA C-acyltransferase - Beta-galactoside transacetylase - Carnitine O-palmitoyltransferase CPT1, CPT2 - Acyltransferase like 2 - Chloramphenicol acetyltransferase - Aminolevulinic acid synthase - Beta-ketoacyl-ACP synthase - Glyceronephosphate O-acyltransferase - Lecithin-cholesterol acyltransferase - Histone acetyltransferase P300/CBP - Serotonin N-acetyl transferase - Serine C-palmitoyltransferase SPTLC1, SPTLC2 - HGSNAT 2.3.2 - Aminoacyltransferases Gamma-glutamyl transpeptidase - Peptidyl transferase - Transglutaminase Tissue transglutaminase, Keratinocyte transglutaminase, Factor XIII 2.3.3 - converted into alkyl on transfer Citrate synthase - ATP citrate lyase - HMG-CoA synthase v d e Metabolism: lipid metabolism - triglyceride and fatty acid enzymes Lipid production ATP citrate lyase - Acetyl-CoA carboxylase Fatty acid synthesis/Fatty acid synthase: Beta-ketoacyl-ACP synthase - Β-Ketoacyl ACP reductase - 3-Hydroxyacyl ACP dehydrase - Enoyl ACP reductase triacyl glycerol: Glycerol-3-phosphate dehydrogenase - Thiokinase Lipid degradation Acyl transport: Carnitine palmitoyltransferase I - Carnitine-acylcarnitine translocase - Carnitine palmitoyltransferase II Beta oxidation/Mitochondrial trifunctional protein: Acyl CoA dehydrogenase ACADL, ACADM, ACADS, ACADVL - Enoyl-CoA hydratase - 3-Hydroxyacyl CoA dehydrogenase - Acetyl-CoA C-acyltransferase to acetyl-CoA: Malonyl-CoA decarboxylase unsaturated Enoyl CoA isomerase, 2,4 Dienoyl-CoA reductase odd chain Propionyl-CoA carboxylase aldehydes Long-chain-aldehyde dehydrogenase see also disorders v d e Mitochondrial enzymes and transporters Outer membrane fatty acid degradation Carnitine palmitoyltransferase I, Long fatty acyl CoA synthetase tryptophan metabolism Kynureninase monoamine neurotransmitter metabolism Monoamine oxidase Intermembrane space Adenylate kinase - Creatine kinase Inner membrane oxidative phosphorylation Coenzyme Q - cytochrome c reductase, Cytochrome c, NADH dehydrogenase, Succinate dehydrogenase pyrimidine metabolism Dihydroorotate dehydrogenase mitochondrial shuttle Malate-aspartate shuttle, Glycerol phosphate shuttle other Glutamate aspartate transporter, Glycerol-3-phosphate dehydrogenase, ATP synthase, Carnitine palmitoyltransferase II Matrix citric acid cycle Citrate synthase, Aconitase, Isocitrate dehydrogenase, Oxoglutarate dehydrogenase, Succinyl coenzyme A synthetase, Fumarase, Malate dehydrogenase anaplerotic reactions Aspartate transaminase, Glutamate dehydrogenase, Pyruvate dehydrogenase complex urea cycle Carbamoyl phosphate synthetase I, Ornithine transcarbamylase, N-Acetylglutamate synthase alcohol metabolism ALDH2 Other/to be sorted Cholesterol side-chain cleavage enzyme Mitochondrial DNA Complex I 7 units - Complex III 1 unit - Complex IV 3 units - ATP synthase 2 units Retrieved from http://en..org/wiki/Carnitine_palmitoyltransferase_II Categories: Genes on chromosome 1 | Human proteins | Chromosome 1 gene stubsHidden category: Protein pages needing a picture Views Article Discussion this page History Personal tools Log in / create account Navigation Main page Contents Featured content Current events Random article Search Go Search Interaction Community portal Recent changes Contact Donate to Help Toolbox What links here Related changes Upload file Special pages Printable version Permanent link Cite this page This page was last modified on 8 July 2008, at 04:35
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