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Carnitine palmitoyltransferase I also known as carnitine acyltransferase I, CPTI, CAT1, CoA:carnitine acyl transferase , 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 , 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.

CPT1A
Available structures
PDB Ortholog search: PDBe RCSB
Identifiers
Aliases CPT1A, carnitine palmitoyltransferase 1A (liver), CPT1, CPT1-L, L-CPT1, carnitine palmitoyltransferase 1A
External IDs MGI: 1098296 HomoloGene: 1413 GeneCards: CPT1A
Genetically Related Diseases
obesity, lipid metabolism disorder
Orthologs
Species Human Mouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

NM_001031847
NM_001876

NM_013495

RefSeq (protein)

NP_001027017.1
NP_001867.2

NP_038523.2

Location (UCSC) Chr 11: 68.75 – 68.84 Mb Chr 19: 3.32 – 3.39 Mb
PubMed search
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.