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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).

Enzymes

3252
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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
3.2.1.21
Inhibitors
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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How to cite this family page

Database page citation:

Carnitine palmitoyltransferases. Accessed on 23/02/2024. IUPHAR/BPS Guide to PHARMACOLOGY, http://www.guidetopharmacology.org/GRAC/FamilyDisplayForward?familyId=1089.

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.