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Carnitine palmitoyltransferases

Unless otherwise stated all data on this page refer to the human proteins. Gene information is provided for human (Hs), mouse (Mm) and rat (Rn).


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carnitine palmitoyltransferase 1A Show summary »

carnitine palmitoyltransferase 1B Show summary »

carnitine palmitoyltransferase 1C Show summary »

carnitine palmitoyltransferase 2 Show summary »

Target Id 3252
Nomenclature carnitine palmitoyltransferase 2
Previous and unofficial names carnitine O-palmitoyltransferase 2 | Carnitine O-palmitoyltransferase 2, mitochondrial
Genes CPT2 (Hs), Cpt2 (Mm), Cpt2 (Rn)
Ensembl ID ENSG00000157184 (Hs), ENSMUSG00000028607 (Mm), ENSRNOG00000012443 (Rn)
UniProtKB AC P23786 (Hs), P52825 (Mm), P18886 (Rn)
EC number
compound 16 [PMID: 17585909] pIC50 6.7 [3] - Rat
teglicar pIC50 6.6 [4] - Rat
Comment Loss of CPT2 function causes the rare lipid metabolism disorder carnitine palmitoyltransferase II deficiency, where long-chain fatty acids cannot be transported into mitochondria for processing and energy production via β-oxidation. The disorder can manifest as neonatal, infantile and adult forms which differ in their tissue-specific symptoms. The 3D stuctures of rat CPT2 in complex with teglicar (PDB ID 2FW3) [4] and a palmitoyl-carnitine substrate analogue (PDB ID 2RCU) [3] have been resolved.


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Further reading

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How to cite this family page

Database page citation:

Carnitine palmitoyltransferases. Accessed on 23/02/2024. IUPHAR/BPS Guide to PHARMACOLOGY,

Concise Guide to PHARMACOLOGY citation:

Alexander SPH, Fabbro D, Kelly E, Mathie AA, Peters JA, Veale EL, Armstrong JF, Faccenda E, Harding SD, Davies JA et al. (2023) The Concise Guide to PHARMACOLOGY 2023/24: Enzymes. Br J Pharmacol. 180 Suppl 2:S289-373.