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
Acyl-CoA from cytosol to the mitochondrial matrix.gif

See also

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