Carnitine palmitoyltransferase I

CPT1A
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	2LE3
Identifiers
Aliases	CPT1A, carnitine palmitoyltransferase 1A (liver), CPT1, CPT1-L, L-CPT1, carnitine palmitoyltransferase 1A
External IDs	OMIM: 600528 MGI: 1098296 HomoloGene: 1413 GeneCards: CPT1A
Gene location (Human)
Chr.	Chromosome 11 (human)
End	68,844,410 bp
Gene location (Mouse)
Chr.	Chromosome 19 (mouse)
End	3,435,733 bp
RNA expression pattern
	Top expressed in
	jejunal mucosa; ; renal medulla; ; duodenum; ; rectum; ; spongy bone; ; smooth muscle tissue; ; gastric mucosa; ; monocyte; ; trigeminal ganglion; ; right ventricle;
	Top expressed in
	gallbladder; ; kidney; ; external carotid artery; ; adrenal gland; ; left lobe of liver; ; epithelium of stomach; ; submandibular gland; ; pyloric antrum; ; cumulus cell; ; large intestine;
	More reference expression data
	n/a
Gene ontology
Molecular function	transferase activity; carnitine O-palmitoyltransferase activity; palmitoleoyltransferase activity; identical protein binding; acyltransferase activity;
Cellular component	integral component of membrane; membrane; intracellular membrane-bounded organelle; integral component of mitochondrial outer membrane; mitochondrial outer membrane; mitochondrion; mitochondrial inner membrane;
Biological process	carnitine shuttle; positive regulation of fatty acid beta-oxidation; epithelial cell differentiation; response to organic cyclic compound; eating behavior; regulation of fatty acid oxidation; regulation of insulin secretion; lipid metabolism; fatty acid metabolic process; cellular response to fatty acid; circadian rhythm; glucose metabolic process; fatty acid beta-oxidation; long-chain fatty acid metabolic process; protein homooligomerization; triglyceride metabolic process; carnitine metabolic process; regulation of lipid metabolic process; transport;
	Sources:Amigo / QuickGO
Orthologs
	1374
	12894
	ENSG00000110090
	ENSMUSG00000024900
	P50416
	P97742
	NM_001031847; NM_001876
	NM_013495
	NP_001027017; NP_001867
	NP_038523
	Wikidata
View/Edit Human	View/Edit Mouse

Carnitine palmitoyltransferase I (CPT1) also known as carnitine acyltransferase I, CPTI, CAT1, CoA:carnitine acyl transferase (CCAT), or palmitoylCoA transferase I, is a mitochondrial enzyme responsible for the formation of acyl carnitines by catalyzing the transfer of the acyl group of a long-chain fatty acyl-CoA from coenzyme A to l-carnitine. The product is often Palmitoylcarnitine (thus the name), but other fatty acids may also be substrates. It is part of a family of enzymes called carnitine acyltransferases. This "preparation" allows for subsequent movement of the acyl carnitine from the cytosol into the intermembrane space of mitochondria.

Three isoforms of CPT1 are currently known: CPT1A, CPT1B, and CPT1C. CPT1 is associated with the outer mitochondrial membrane. This enzyme can be inhibited by malonyl CoA, the first committed intermediate produced during fatty acid synthesis. Its role in fatty acid metabolism makes CPT1 important in many metabolic disorders such as diabetes. Since its crystal structure is not known, its exact mechanism of action remains to be determined.

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