Carnitine O-palmitoyltransferase

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

Molecules

Palmitoylcarnitine
Palmitoyl CoA

Pathway

Acyl-CoA from cytosol to the mitochondrial matrix

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

References

↑ Jogl G, Tong L (January 2003). "Crystal structure of carnitine acetyltransferase and implications for the catalytic mechanism and fatty acid transport". Cell 112 (1): 113–22. doi:10.1016/S0092-8674(02)01228-X. PMID 12526798.

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
Carnitine O-Palmitoyltransferase at the US National Library of Medicine Medical Subject Headings (MeSH)

Transferases: acyltransferases (EC 2.3)

2.3.1: other than amino-acyl groups

acetyltransferases: Acetyl-Coenzyme A acetyltransferase
N-Acetylglutamate synthase
Choline acetyltransferase
Dihydrolipoyl transacetylase
Acetyl-CoA C-acyltransferase
Beta-galactoside transacetylase
Chloramphenicol acetyltransferase
N-acetyltransferase
- Serotonin N-acetyl transferase
- HGSNAT
- ARD1A
Histone acetyltransferase
- P300/CBP
- NAT2

palmitoyltransferases: Carnitine O-palmitoyltransferase
- CPT1
- CPT2
Serine C-palmitoyltransferase
- SPTLC1
- SPTLC2

2.3.2: Aminoacyltransferases

2.3.3: converted into alkyl on transfer

B
enzm
1.1
- 2
- 3
- 4
- 5
- 6
- 7
- 8
- 10
- 11
- 13
- 14
- 15-18
2.1
- 2
- 3
- 4
- 5
- 6
- 7
  - 2.7.10
  - 11-12
- 8
3.1
- - 3.1.3.48
- 2
- 3
- 4
  - 3.4.21
  - 22
  - 23
  - 24
- 5
- 6
- 7
4.1
- 2
- 3
- 4
- 5
- 6
5.1
- 2
- 3
- 4
- 99
6.1-3
- 4
- 5-6

Metabolism: lipid metabolism / fatty acid metabolism, triglyceride and fatty acid enzymes

Synthesis

Malonyl-CoA synthesis	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

Fatty acid desaturases	Stearoyl-CoA desaturase-1

Triacyl glycerol	Glycerol-3-phosphate dehydrogenase Thiokinase

Degradation

Acyl transport

Beta oxidation

General	Acyl CoA dehydrogenase (ACADL ACADM ACADS ACADVL ACADSB) Enoyl-CoA hydratase MTP: HADH HADHA HADHB Acetyl-CoA C-acyltransferase

Unsaturated	Enoyl CoA isomerase 2,4 Dienoyl-CoA reductase

Odd chain	Propionyl-CoA carboxylase

Other	Hydroxyacyl-Coenzyme A dehydrogenase

To acetyl-CoA

Malonyl-CoA decarboxylase

Aldehydes

Long-chain-aldehyde dehydrogenase

M: MET

mt, k, c/g/r/p/y/i, f/h/s/l/o/e, a/u, n, m

k, cgrp/y/i, f/h/s/l/o/e, au, n, m, epon

m (A16/C10), i (k, c/g/r/p/y/i, f/h/s/o/e, a/u, n, m)

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