Carnitine O-palmitoyltransferase

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Carnitine O-palmitoyltransferase (also called carnitine palmitoyltransferase or carnitine acyltransferase) is a mitochondrial transferase enzyme (EC 2.3.1.21) involved in the metabolism of palmitoylcarnitine into palmitoyl-CoA.

Contents 1 Molecules 2 Pathway 3 Human forms of CPT 4 See also 5 External links

[edit] Molecules

Palmitoylcarnitine

Palmitoyl CoA

[edit] Pathway

[edit] Human forms of CPT

There are four different forms of CPT in humans:

• CPT1A - associated with Carnitine palmitoyltransferase I deficiency
• CPT1B
• CPT1C
• CPT2 - associated with Carnitine palmitoyltransferase II deficiency

[edit] See also

• Fatty_acid_degradation#Transport_into_the_mitochondrial_matrix

[edit] External links

• PDOC00402 - Acyltransferases ChoActase / COT / CPT family in PROSITE
• Choline/Carnitine o-acyltransferase family in Pfam
• UMich Orientation of Proteins in Membranes protein/pdbid-2h4t
• MeSH Carnitine+O-Palmitoyltransferase

v • d • e Transferases: acyltransferases (EC 2.3) 2.3.1: other than amino-acyl groups (mostly acetyltransferases) N-Acetylglutamate synthase - Choline acetyltransferase - Acetyl-Coenzyme A acetyltransferase - Dihydrolipoyl transacetylase - Acetyl-CoA C-acyltransferase - Beta-galactoside transacetylase - Carnitine O-palmitoyltransferase (CPT1, CPT2) - Acyltransferase like 2 - Chloramphenicol acetyltransferase - Aminolevulinic acid synthase - Beta-ketoacyl-ACP synthase - Glyceronephosphate O-acyltransferase - Lecithin-cholesterol acyltransferase - Histone acetyltransferase (P300/CBP) - Serotonin N-acetyl transferase - Serine C-palmitoyltransferase (SPTLC1, SPTLC2) - HGSNAT 2.3.2 - Aminoacyltransferases Gamma-glutamyl transpeptidase - Peptidyl transferase - Transglutaminase (Tissue transglutaminase, Keratinocyte transglutaminase, Factor XIII) 2.3.3 - converted into alkyl on transfer Citrate synthase - ATP citrate lyase - HMG-CoA synthase

v • d • e Metabolism: lipid metabolism - triglyceride and fatty acid enzymes Lipid production ATP citrate lyase - Acetyl-CoA carboxylase Fatty acid synthesis/Fatty acid synthase: Beta-ketoacyl-ACP synthase - Β-Ketoacyl ACP reductase - 3-Hydroxyacyl ACP dehydrase - Enoyl ACP reductase triacyl glycerol: Glycerol-3-phosphate dehydrogenase - Thiokinase Lipid degradation Acyl transport: Carnitine palmitoyltransferase I - Carnitine-acylcarnitine translocase - Carnitine palmitoyltransferase II Beta oxidation/Mitochondrial trifunctional protein: Acyl CoA dehydrogenase (ACADL, ACADM, ACADS, ACADVL) - Enoyl-CoA hydratase - 3-Hydroxyacyl CoA dehydrogenase - Acetyl-CoA C-acyltransferase to acetyl-CoA: Malonyl-CoA decarboxylase unsaturated (Enoyl CoA isomerase, 2,4 Dienoyl-CoA reductase) odd chain (Propionyl-CoA carboxylase) aldehydes (Long-chain-aldehyde dehydrogenase) see also disorders