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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).
carnitine palmitoyltransferase 1A Show summary » More detailed page |
carnitine palmitoyltransferase 1B Show summary » More detailed page |
carnitine palmitoyltransferase 1C Show summary » More detailed page |
carnitine palmitoyltransferase 2 Show summary » More detailed page |
Database page citation:
Carnitine palmitoyltransferases. Accessed on 04/11/2024. 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.
The CPT1 enzymes convert long chain (greater than ∼C12) acyl-CoA molecules to acylcarnitine derivatives to facilitate their transport from the cytosol into the mitochondrial matrix via the carnitine/acylcarnitine carrier (CACT; SLC25A20). The acylcarnitines are converted back to acyl-CoA by CPT2 to support the fatty acid oxidation (FAO) cycle in the mitochondria. CPT1s are allosterically inhibited by malonyl-CoA [5].
Tissue expression: CPT1A is the only CPT1 isoform expressed in the liver and is detected in heart muscle, but is absent from skeletal muscle and adipose tissue. In contrast CPT1B is highly expressed in skeletal and heart muscle, adipose tissue and testis. CPT1C expression is restricted to the brain, and CPT2 is ubiquitously expressed.
Pathophysiology and therapeutic potential: Dysregualtion of CPT activity is associated with serious human diseases. Given their role in FAO the CPT1s are considered as tractable targets for the development of therapeutic agents for the treament of type 2 diabetes and obesity. As a consequence of the dependence of some cancer cells on FAO for energy production, inhibiting CPT1 function offers an attractive mechanism to 'starve' these cells and limit their proliferative potential.