CPT2
CPT2 (carnitine palmitoyltransferase 2, also known as CPTASE and CPT1) is a mitochondrial inner membrane enzyme that reconverts acylcarnitines to acyl-CoA esters for beta-oxidation. It works with carnitine palmitoyltransferase I in the oxidation of long-chain fatty acids. Browse Assay Genie's current range of CPT2 research reagents below, or read on for the biology behind the target.
CPT2 is involved in the intramitochondrial synthesis of acylcarnitines from accumulated acyl-CoA metabolites. Its central role, however, is the reverse reaction: it reconverts acylcarnitines back into their respective acyl-CoA esters, which can then undergo beta-oxidation. This is an essential step in the mitochondrial uptake of long-chain fatty acids and their subsequent beta-oxidation inside the mitochondrion. The enzyme is active with medium-chain (C8-C12) and long-chain (C14-C18) acyl-CoA esters.
The CPT2 gene encodes a protein that is made from a nuclear gene and then transported to the mitochondrial inner membrane. CPT2 belongs to the carnitine/choline acetyltransferase family and sits within the HGNC carnitine palmitoyltransferase family, which also includes CPT1A and CPT1B. Together with carnitine palmitoyltransferase I, CPT2 oxidises long-chain fatty acids in the mitochondria, so the two enzymes act in sequence in the carnitine shuttle pathway. Researchers studying the CPT2 protein often examine it alongside these partner enzymes.
The enzyme has also been called carnitine palmitoyltransferase II, and the symbols CPTASE and CPT1 appear as aliases. Defects in the CPT2 gene are associated with mitochondrial long-chain fatty-acid (LCFA) oxidation disorders, which makes the enzyme a common reference point in studies of fatty acid metabolism and mitochondrial function. Mouse and rat both have a Cpt2 ortholog.
CPT2 reconverts acylcarnitines to acyl-CoA esters, an essential step before beta-oxidation of long-chain fatty acids in the mitochondrion.
As an inner membrane enzyme that handles medium- and long-chain acyl groups, CPT2 is a focus for studies of mitochondrial lipid handling.
Defects in the CPT2 gene are associated with mitochondrial long-chain fatty-acid oxidation disorders, making it relevant to disease-mechanism research.
CPT2 acts with carnitine palmitoyltransferase I, so it is studied alongside CPT1A and CPT1B to understand long-chain fatty acid entry into mitochondria.
CPT2 is involved in making acylcarnitines from acyl-CoA metabolites inside mitochondria, and it reconverts acylcarnitines to acyl-CoA esters that can enter beta-oxidation. This is essential for mitochondrial uptake and oxidation of long-chain fatty acids. It acts on C8-C12 and C14-C18 acyl-CoA esters.
The HGNC name is carnitine palmitoyltransferase 2, previously carnitine palmitoyltransferase II. UniProt lists it as carnitine O-palmitoyltransferase 2, mitochondrial. Aliases include CPTASE and CPT1, which can be confused with the separate CPT1A and CPT1B genes, so check the gene symbol and UniProt accession when searching.
Defects in the CPT2 gene are associated with mitochondrial long-chain fatty-acid oxidation disorders. Mouse and rat each have a Cpt2 ortholog, so rodent models are used in this area. Check the datasheet of any product for its validated reactivity.
Other carnitine palmitoyltransferase family members that act with CPT2 in mitochondrial long-chain fatty acid oxidation.
Gene and protein annotations from HGNC, NCBI Gene and UniProt P23786; product specifications from Assay Genie datasheets.