Carnitine O-palmitoyltransferase
Encyclopedia
Carnitine O-palmitoyltransferase (also called carnitine palmitoyltransferase) is a mitochondrial transferase
enzyme
involved in the metabolism of palmitoylcarnitine
into palmitoyl-CoA. A related transferase is carnitine acyltransferase.
Transferase
In biochemistry, a transferase is an enzyme that catalyzes the transfer of a functional group from one molecule to another . For example, an enzyme that catalyzed this reaction would be a transferase:In this example, A would be the donor, and B would be the acceptor...
enzyme
Enzyme
Enzymes are proteins that catalyze chemical reactions. In enzymatic reactions, the molecules at the beginning of the process, called substrates, are converted into different molecules, called products. Almost all chemical reactions in a biological cell need enzymes in order to occur at rates...
involved in the metabolism of palmitoylcarnitine
Palmitoylcarnitine
Palmitoylcarnitine is an ester derivative of carnitine involved in the metabolism of fatty acids.Carnitine O-palmitoyltransferase breaks it down into palmitoyl CoA....
into palmitoyl-CoA. A related transferase is carnitine acyltransferase.
Pathway
Human forms of CPT
There are four different forms of CPT in humans:- CPT1A - associated with Carnitine palmitoyltransferase I deficiencyCarnitine palmitoyltransferase I deficiencyCarnitine palmitoyltransferase I deficiency is a rare metabolic disorder that prevents the body from converting certain fats called long-chain fatty acids into energy, particularly during periods without food....
- CPT1B
- CPT1C
- CPT2Carnitine palmitoyltransferase IICarnitine O-palmitoyltransferase 2, mitochondrial is an enzyme that in humans is encoded by the CPT2 gene.-Further reading:...
- associated with Carnitine palmitoyltransferase II deficiencyCarnitine palmitoyltransferase II deficiencyCarnitine palmitoyltransferase II deficiency is a metabolic disorder characterized by an enzymatic defect that prevents long-chain fatty acids from being transported into the mitochondria for utilization as an energy source....
External links
- Acyltransferases ChoActase / COT / CPT family in PROSITEPROSITE
PROSITE is a protein database. It consists of entries describing the protein families, domains and functional sites as well as amino acid patterns, signatures, and profiles in them. These are manually curated by a team of the Swiss Institute of Bioinformatics and tightly integrated into Swiss-Prot...
- Choline/Carnitine o-acyltransferase family in PfamPfamPfam is a database of protein families that includes their annotations and multiple sequence alignments generated using hidden Markov models.- Features :For each family in Pfam one can:* Look at multiple alignments* View protein domain architectures...