Carnitine palmitoyltransferase I

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carnitine palmitoyltransferase 1A (liver)
Identifiers
Symbol	CPT1A
Alt. Symbols	CPT1
Entrez	1374
HUGO	2328
OMIM	600528
RefSeq	NM_001876
UniProt	P50416
Other data
EC number	2.3.1.21
Locus	Chr. 11 q13.1-13.5

carnitine palmitoyltransferase 1B (muscle)
Identifiers
Symbol	CPT1B
Entrez	1375
HUGO	2329
OMIM	601987
RefSeq	NM_152245
UniProt	Q92523
Other data
Locus	Chr. 22 q13.3

carnitine palmitoyltransferase 1C
Identifiers
Symbol	CPT1C
Entrez	126129
HUGO	18540
OMIM	608846
RefSeq	NM_152359
UniProt	Q8TCG5
Other data
Locus	Chr. 19 q13.33

Carnitine palmitoyltransferase I (CPT1)is a mitochondrial enzyme. In muscle and other non-liver tissues, CPT1 is associated with the outer mitochondrial membrane. CPT1 mediates the transport of long chain fatty acids across the membrane by binding them to carnitine. This enzyme can be inhibited by malonyl CoA.

The "CPT1A" form is associated with carnitine palmitoyltransferase I deficiency.

Acyl-CoA from cytosol to the mitochondrial matrix.gif

[edit] See also

Carnitine palmitoyltransferase II

This transferase article is a stub. You can help Wikipedia by expanding it.

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 Mitochondrial enzymes and transporters

Outer membrane	fatty acid degradation (Carnitine palmitoyltransferase I, Long fatty acyl CoA synthetase) tryptophan metabolism (Kynureninase) monoamine neurotransmitter metabolism (Monoamine oxidase)

Intermembrane space	Adenylate kinase - Creatine kinase

Inner membrane	oxidative phosphorylation (Coenzyme Q - cytochrome c reductase, Cytochrome c, NADH dehydrogenase, Succinate dehydrogenase) pyrimidine metabolism (Dihydroorotate dehydrogenase) mitochondrial shuttle (Malate-aspartate shuttle, Glycerol phosphate shuttle) other (Glutamate aspartate transporter, Glycerol-3-phosphate dehydrogenase, ATP synthase, Carnitine palmitoyltransferase II)

Matrix	citric acid cycle (Citrate synthase, Aconitase, Isocitrate dehydrogenase, Oxoglutarate dehydrogenase, Succinyl coenzyme A synthetase, Fumarase, Malate dehydrogenase) anaplerotic reactions (Aspartate transaminase, Glutamate dehydrogenase, Pyruvate dehydrogenase complex) urea cycle (Carbamoyl phosphate synthetase I, Ornithine transcarbamylase, N-Acetylglutamate synthase) alcohol metabolism (ALDH2)

Other/to be sorted	Cholesterol side-chain cleavage enzyme

Mitochondrial DNA	Complex I (7 units) - Complex III (1 unit) - Complex IV (3 units) - ATP synthase (2 units)