2.3.1.21

carnitine O-palmitoyltransferase

carnitine palmitoyltransferase 1a

fatty acid metabolism/fatty acid oxidation

Mouse (10090)

7 B2[78070 ], 15 E3|15 52.6 cM[12895 ], 4 C7|4 54.4 cM[12896 ], 19 A|19 2.0 cM[12894 ],

Broad specificity to acyl group, over the range C8 to C18; optimal activity with palmitoyl-CoA. cf. EC 2.3.1.7 carnitine O-acetyltransferase and EC 2.3.1.137 carnitine O-octanoyltransferase.

"To clarify the paradoxic effects of cerulenin, namely its in vitro inhibitory effects on fat catabolism and its in vivo reduction of fat mass, we studied the in vivo and in vitro effects of cerulenin on carnitine palmitoyltransferase-1 (CPT-1) activity, the rate-limiting enzyme of fatty acid oxidation." (15044358)

15378;12912;19013

phosphorylation;

phosphorylation;

phosphorylation;

phosphorylation;

phosphorylation;

phosphorylation;

transcriptional factor;"CREB(12912),HNF4alpha(15378)"

"Results show that CREB and HNF4alpha bind the CPT promoter, that PGC-1 is involved in liver carnitine palmitoyltransferase I (CPT) gene activation by cylic AMP, and that PGC-1 acts to coordinate the process of metabolic adaptation in the liver." (12107181)

transcriptional factor;PPARalpha(19013)

"The PPARalpha targeted genes CPT-II and HMG-CoA synthase are potential regulators of mitochondrial fatty acid oxidation and ketogenesis in hTNFalpha transgenic mice, and the increased propionyl-CoA level found is a possible inhibitor of these processes. " (15893958)

GO:0006810 (P) transport [P52825 ];
GO:0004095 (F) carnitine O-palmitoyltransferase activity [Q924X2, P52825, P97742, Q8BGD5 ];
GO:0006631 (P) fatty acid metabolic process [Q924X2, P52825, P97742, Q8BGD5 ];
GO:0016021 (C) integral to membrane [Q924X2, P97742, Q8BGD5 ];
GO:0015909 (P) long-chain fatty acid transport [Q924X2 ];
GO:0005741 (C) mitochondrial outer membrane [Q924X2, P97742, Q8BGD5 ];
GO:0005743 (C) mitochondrial inner membrane [P52825, P97742 ];

mitochondrion [Q924X2, P52825, P97742, Q8BGD5 ];

membrane [P52825 ];

P52825; P97742; Q8BGD5; Q924X2

12894; 12895; 12896; 78070