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Showing 49821 - 49830 of 605359 pathways
SMPDB ID Pathway Name and Description Pathway Class Chemical Compounds Proteins

SMP0124353

Pw125809 View Pathway

Acylcarnitine 11-HydroxyNonadecanoylcarnitine

11-HydroxyNonadecanoylcarnitine is an acylcarnitine. The general role of acylcarnitines is to transport acyl-groups, organic acids and fatty acids, from the cytoplasm into the mitochondria so that they can be broken down to produce energy. As part of this process, 11-hydroxyNonadecanoic acid is first transported into the cell via the long-chain fatty acid transport protein 1 (FATP1). Once inside the cell it undergoes a reaction to form an acyl-CoA derivative called 11-hydroxyNonadecanoyl-CoA. This reaction is facilitated by the long-chain fatty-acid CoA ligase 1 protein, which adds a CoA moiety to appropriate acyl groups. Many acyl-CoA groups will then further react with other zwitterionic compounds such as carnitine (to form acylcarnitines) and amino acids (to form acyl amides). The carnitine needed to form acylcarnitines inside the cell is transported into the cell by the organic cation/carnitine transporter 2. In forming an acylcarnitine derivative, 11-hydroxyNonadecanoyl-CoA reacts with L-carnitine to form 11-hydroxyNonadecanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 11-hydroxyNonadecanoylcarnitine is moved into the mitochondrial intermembrane space. Following the reaction, the newly synthesized acylcarnitine is transported into the mitochondrial matrix by a mitochondrial carnitine/acylcarnitine carrier protein found in the mitochondrial inner membrane. Once in the matrix, 11-hydroxyNonadecanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 11-hydroxyNonadecanoyl-CoA and L-carnitine. 11-HydroxyNonadecanoyl-CoA then enters into the mitochondrial beta-oxidation pathway to form aceytl-CoA. Acetyl-CoA can go on to enter the TCA cycle, or it can react with L-carnitine to form L-acetylcarnitine in a reaction catalyzed by Carnitine O-acetyltransferase. This reaction can occur in both directions, and L-acetylcarnitine and CoA can react to form acetyl-CoA and L-carnitine in certain circumstances. Finally, acetyl-CoA in the cytosol can be catalyzed by acetyl-CoA carboxylase 1 to form malonyl-CoA, which inhibits the action of carnitine O-palmitoyltransferase 1, thereby preventing 11-hydroxyNonadecanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolic

SMP0124355

Pw125811 View Pathway

Acylcarnitine (9Z)-Nonadec-9-enoylcarnitine

(9Z)-Nonadec-9-enoylcarnitine is an acylcarnitine. The general role of acylcarnitines is to transport acyl-groups, organic acids and fatty acids, from the cytoplasm into the mitochondria so that they can be broken down to produce energy. As part of this process, (9Z)-Nonadec-9-enoic acid is first transported into the cell via the long-chain fatty acid transport protein 1 (FATP1). Once inside the cell it undergoes a reaction to form an acyl-CoA derivative called (9Z)-Nonadec-9-enoyl-CoA. This reaction is facilitated by the long-chain fatty-acid CoA ligase 1 protein, which adds a CoA moiety to appropriate acyl groups. Many acyl-CoA groups will then further react with other zwitterionic compounds such as carnitine (to form acylcarnitines) and amino acids (to form acyl amides). The carnitine needed to form acylcarnitines inside the cell is transported into the cell by the organic cation/carnitine transporter 2. In forming an acylcarnitine derivative, (9Z)-Nonadec-9-enoyl-CoA reacts with L-carnitine to form (9Z)-Nonadec-9-enoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (9Z)-Nonadec-9-enoylcarnitine is moved into the mitochondrial intermembrane space. Following the reaction, the newly synthesized acylcarnitine is transported into the mitochondrial matrix by a mitochondrial carnitine/acylcarnitine carrier protein found in the mitochondrial inner membrane. Once in the matrix, (9Z)-Nonadec-9-enoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (9Z)-Nonadec-9-enoyl-CoA and L-carnitine. (9Z)-Nonadec-9-enoyl-CoA then enters into the mitochondrial beta-oxidation pathway to form aceytl-CoA. Acetyl-CoA can go on to enter the TCA cycle, or it can react with L-carnitine to form L-acetylcarnitine in a reaction catalyzed by Carnitine O-acetyltransferase. This reaction can occur in both directions, and L-acetylcarnitine and CoA can react to form acetyl-CoA and L-carnitine in certain circumstances. Finally, acetyl-CoA in the cytosol can be catalyzed by acetyl-CoA carboxylase 1 to form malonyl-CoA, which inhibits the action of carnitine O-palmitoyltransferase 1, thereby preventing (9Z)-Nonadec-9-enoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolic

SMP0124360

Pw125816 View Pathway

Acylcarnitine 2-hydroxyicosanoylcarnitine

2-hydroxyicosanoylcarnitine is an acylcarnitine. The general role of acylcarnitines is to transport acyl-groups, organic acids and fatty acids, from the cytoplasm into the mitochondria so that they can be broken down to produce energy. As part of this process, 2-hydroxyicosanoic acid is first transported into the cell via the long-chain fatty acid transport protein 1 (FATP1). Once inside the cell it undergoes a reaction to form an acyl-CoA derivative called 2-hydroxyicosanoyl-CoA. This reaction is facilitated by the long-chain fatty-acid CoA ligase 1 protein, which adds a CoA moiety to appropriate acyl groups. Many acyl-CoA groups will then further react with other zwitterionic compounds such as carnitine (to form acylcarnitines) and amino acids (to form acyl amides). The carnitine needed to form acylcarnitines inside the cell is transported into the cell by the organic cation/carnitine transporter 2. In forming an acylcarnitine derivative, 2-hydroxyicosanoyl-CoA reacts with L-carnitine to form 2-hydroxyicosanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 2-hydroxyicosanoylcarnitine is moved into the mitochondrial intermembrane space. Following the reaction, the newly synthesized acylcarnitine is transported into the mitochondrial matrix by a mitochondrial carnitine/acylcarnitine carrier protein found in the mitochondrial inner membrane. Once in the matrix, 2-hydroxyicosanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 2-hydroxyicosanoyl-CoA and L-carnitine. 2-hydroxyicosanoyl-CoA then enters into the mitochondrial beta-oxidation pathway to form aceytl-CoA. Acetyl-CoA can go on to enter the TCA cycle, or it can react with L-carnitine to form L-acetylcarnitine in a reaction catalyzed by Carnitine O-acetyltransferase. This reaction can occur in both directions, and L-acetylcarnitine and CoA can react to form acetyl-CoA and L-carnitine in certain circumstances. Finally, acetyl-CoA in the cytosol can be catalyzed by acetyl-CoA carboxylase 1 to form malonyl-CoA, which inhibits the action of carnitine O-palmitoyltransferase 1, thereby preventing 2-hydroxyicosanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolic

SMP0124405

Pw125861 View Pathway

Acylcarnitine (5Z,7S,8E,10Z,13Z,15E,17S,19Z)-7,17-dihydroxydocosa-5,8,10,13,15,19-hexaenoylcarnitine

(5Z,7S,8E,10Z,13Z,15E,17S,19Z)-7,17-dihydroxydocosa-5,8,10,13,15,19-hexaenoylcarnitine is an acylcarnitine. The general role of acylcarnitines is to transport acyl-groups, organic acids and fatty acids, from the cytoplasm into the mitochondria so that they can be broken down to produce energy. As part of this process, (5Z,7S,8E,10Z,13Z,15E,17S,19Z)-7,17-dihydroxydocosa-5,8,10,13,15,19-hexaenoic acid is first transported into the cell via the long-chain fatty acid transport protein 1 (FATP1). Once inside the cell it undergoes a reaction to form an acyl-CoA derivative called (5Z,7S,8E,10Z,13Z,15E,17S,19Z)-7,17-dihydroxydocosa-5,8,10,13,15,19-hexaenoyl-CoA. This reaction is facilitated by the long-chain fatty-acid CoA ligase 1 protein, which adds a CoA moiety to appropriate acyl groups. Many acyl-CoA groups will then further react with other zwitterionic compounds such as carnitine (to form acylcarnitines) and amino acids (to form acyl amides). The carnitine needed to form acylcarnitines inside the cell is transported into the cell by the organic cation/carnitine transporter 2. In forming an acylcarnitine derivative, (5Z,7S,8E,10Z,13Z,15E,17S,19Z)-7,17-dihydroxydocosa-5,8,10,13,15,19-hexaenoyl-CoA reacts with L-carnitine to form (5Z,7S,8E,10Z,13Z,15E,17S,19Z)-7,17-dihydroxydocosa-5,8,10,13,15,19-hexaenoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (5Z,7S,8E,10Z,13Z,15E,17S,19Z)-7,17-dihydroxydocosa-5,8,10,13,15,19-hexaenoylcarnitine is moved into the mitochondrial intermembrane space. Following the reaction, the newly synthesized acylcarnitine is transported into the mitochondrial matrix by a mitochondrial carnitine/acylcarnitine carrier protein found in the mitochondrial inner membrane. Once in the matrix, (5Z,7S,8E,10Z,13Z,15E,17S,19Z)-7,17-dihydroxydocosa-5,8,10,13,15,19-hexaenoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (5Z,7S,8E,10Z,13Z,15E,17S,19Z)-7,17-dihydroxydocosa-5,8,10,13,15,19-hexaenoyl-CoA and L-carnitine. (5Z,7S,8E,10Z,13Z,15E,17S,19Z)-7,17-dihydroxydocosa-5,8,10,13,15,19-hexaenoyl-CoA then enters into the mitochondrial beta-oxidation pathway to form aceytl-CoA. Acetyl-CoA can go on to enter the TCA cycle, or it can react with L-carnitine to form L-acetylcarnitine in a reaction catalyzed by Carnitine O-acetyltransferase. This reaction can occur in both directions, and L-acetylcarnitine and CoA can react to form acetyl-CoA and L-carnitine in certain circumstances. Finally, acetyl-CoA in the cytosol can be catalyzed by acetyl-CoA carboxylase 1 to form malonyl-CoA, which inhibits the action of carnitine O-palmitoyltransferase 1, thereby preventing (5Z,7S,8E,10Z,13Z,15E,17S,19Z)-7,17-dihydroxydocosa-5,8,10,13,15,19-hexaenoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolic

SMP0124410

Pw125866 View Pathway

Acylcarnitine (4Z,7E,9E,13Z,16Z,19Z)-11-hydroxydocosa-4,7,9,13,16,19-hexaenoylcarnitine

(4Z,7E,9E,13Z,16Z,19Z)-11-hydroxydocosa-4,7,9,13,16,19-hexaenoylcarnitine is an acylcarnitine. The general role of acylcarnitines is to transport acyl-groups, organic acids and fatty acids, from the cytoplasm into the mitochondria so that they can be broken down to produce energy. As part of this process, (4Z,7E,9E,13Z,16Z,19Z)-11-hydroxydocosa-4,7,9,13,16,19-hexaenoic acid is first transported into the cell via the long-chain fatty acid transport protein 1 (FATP1). Once inside the cell it undergoes a reaction to form an acyl-CoA derivative called (4Z,7E,9E,13Z,16Z,19Z)-11-hydroxydocosa-4,7,9,13,16,19-hexaenoyl-CoA. This reaction is facilitated by the long-chain fatty-acid CoA ligase 1 protein, which adds a CoA moiety to appropriate acyl groups. Many acyl-CoA groups will then further react with other zwitterionic compounds such as carnitine (to form acylcarnitines) and amino acids (to form acyl amides). The carnitine needed to form acylcarnitines inside the cell is transported into the cell by the organic cation/carnitine transporter 2. In forming an acylcarnitine derivative, (4Z,7E,9E,13Z,16Z,19Z)-11-hydroxydocosa-4,7,9,13,16,19-hexaenoyl-CoA reacts with L-carnitine to form (4Z,7E,9E,13Z,16Z,19Z)-11-hydroxydocosa-4,7,9,13,16,19-hexaenoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (4Z,7E,9E,13Z,16Z,19Z)-11-hydroxydocosa-4,7,9,13,16,19-hexaenoylcarnitine is moved into the mitochondrial intermembrane space. Following the reaction, the newly synthesized acylcarnitine is transported into the mitochondrial matrix by a mitochondrial carnitine/acylcarnitine carrier protein found in the mitochondrial inner membrane. Once in the matrix, (4Z,7E,9E,13Z,16Z,19Z)-11-hydroxydocosa-4,7,9,13,16,19-hexaenoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (4Z,7E,9E,13Z,16Z,19Z)-11-hydroxydocosa-4,7,9,13,16,19-hexaenoyl-CoA and L-carnitine. (4Z,7E,9E,13Z,16Z,19Z)-11-hydroxydocosa-4,7,9,13,16,19-hexaenoyl-CoA then enters into the mitochondrial beta-oxidation pathway to form aceytl-CoA. Acetyl-CoA can go on to enter the TCA cycle, or it can react with L-carnitine to form L-acetylcarnitine in a reaction catalyzed by Carnitine O-acetyltransferase. This reaction can occur in both directions, and L-acetylcarnitine and CoA can react to form acetyl-CoA and L-carnitine in certain circumstances. Finally, acetyl-CoA in the cytosol can be catalyzed by acetyl-CoA carboxylase 1 to form malonyl-CoA, which inhibits the action of carnitine O-palmitoyltransferase 1, thereby preventing (4Z,7E,9E,13Z,16Z,19Z)-11-hydroxydocosa-4,7,9,13,16,19-hexaenoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolic

SMP0124403

Pw125859 View Pathway

Acylcarnitine (4Z,7Z,10Z,13Z,16Z)-19,20-dihydroxydocosa-4,7,10,13,16-pentaenoylcarnitine

(4Z,7Z,10Z,13Z,16Z)-19,20-dihydroxydocosa-4,7,10,13,16-pentaenoylcarnitine is an acylcarnitine. The general role of acylcarnitines is to transport acyl-groups, organic acids and fatty acids, from the cytoplasm into the mitochondria so that they can be broken down to produce energy. As part of this process, (4Z,7Z,10Z,13Z,16Z)-19,20-dihydroxydocosa-4,7,10,13,16-pentaenoic acid is first transported into the cell via the long-chain fatty acid transport protein 1 (FATP1). Once inside the cell it undergoes a reaction to form an acyl-CoA derivative called (4Z,7Z,10Z,13Z,16Z)-19,20-dihydroxydocosa-4,7,10,13,16-pentaenoyl-CoA. This reaction is facilitated by the long-chain fatty-acid CoA ligase 1 protein, which adds a CoA moiety to appropriate acyl groups. Many acyl-CoA groups will then further react with other zwitterionic compounds such as carnitine (to form acylcarnitines) and amino acids (to form acyl amides). The carnitine needed to form acylcarnitines inside the cell is transported into the cell by the organic cation/carnitine transporter 2. In forming an acylcarnitine derivative, (4Z,7Z,10Z,13Z,16Z)-19,20-dihydroxydocosa-4,7,10,13,16-pentaenoyl-CoA reacts with L-carnitine to form (4Z,7Z,10Z,13Z,16Z)-19,20-dihydroxydocosa-4,7,10,13,16-pentaenoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (4Z,7Z,10Z,13Z,16Z)-19,20-dihydroxydocosa-4,7,10,13,16-pentaenoylcarnitine is moved into the mitochondrial intermembrane space. Following the reaction, the newly synthesized acylcarnitine is transported into the mitochondrial matrix by a mitochondrial carnitine/acylcarnitine carrier protein found in the mitochondrial inner membrane. Once in the matrix, (4Z,7Z,10Z,13Z,16Z)-19,20-dihydroxydocosa-4,7,10,13,16-pentaenoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (4Z,7Z,10Z,13Z,16Z)-19,20-dihydroxydocosa-4,7,10,13,16-pentaenoyl-CoA and L-carnitine. (4Z,7Z,10Z,13Z,16Z)-19,20-dihydroxydocosa-4,7,10,13,16-pentaenoyl-CoA then enters into the mitochondrial beta-oxidation pathway to form aceytl-CoA. Acetyl-CoA can go on to enter the TCA cycle, or it can react with L-carnitine to form L-acetylcarnitine in a reaction catalyzed by Carnitine O-acetyltransferase. This reaction can occur in both directions, and L-acetylcarnitine and CoA can react to form acetyl-CoA and L-carnitine in certain circumstances. Finally, acetyl-CoA in the cytosol can be catalyzed by acetyl-CoA carboxylase 1 to form malonyl-CoA, which inhibits the action of carnitine O-palmitoyltransferase 1, thereby preventing (4Z,7Z,10Z,13Z,16Z)-19,20-dihydroxydocosa-4,7,10,13,16-pentaenoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolic

SMP0124348

Pw125804 View Pathway

Acylcarnitine (10E,12E,14E)-16-hydroxy-9-oxooctadeca-10,12,14-trienoylcarnitine

(10E,12E,14E)-16-hydroxy-9-oxooctadeca-10,12,14-trienoylcarnitine is an acylcarnitine. The general role of acylcarnitines is to transport acyl-groups, organic acids and fatty acids, from the cytoplasm into the mitochondria so that they can be broken down to produce energy. As part of this process, (10E,12E,14E)-16-hydroxy-9-oxooctadeca-10,12,14-trienoic acid is first transported into the cell via the long-chain fatty acid transport protein 1 (FATP1). Once inside the cell it undergoes a reaction to form an acyl-CoA derivative called (10E,12E,14E)-16-hydroxy-9-oxooctadeca-10,12,14-trienoyl-CoA. This reaction is facilitated by the long-chain fatty-acid CoA ligase 1 protein, which adds a CoA moiety to appropriate acyl groups. Many acyl-CoA groups will then further react with other zwitterionic compounds such as carnitine (to form acylcarnitines) and amino acids (to form acyl amides). The carnitine needed to form acylcarnitines inside the cell is transported into the cell by the organic cation/carnitine transporter 2. In forming an acylcarnitine derivative, (10E,12E,14E)-16-hydroxy-9-oxooctadeca-10,12,14-trienoyl-CoA reacts with L-carnitine to form (10E,12E,14E)-16-hydroxy-9-oxooctadeca-10,12,14-trienoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (10E,12E,14E)-16-hydroxy-9-oxooctadeca-10,12,14-trienoylcarnitine is moved into the mitochondrial intermembrane space. Following the reaction, the newly synthesized acylcarnitine is transported into the mitochondrial matrix by a mitochondrial carnitine/acylcarnitine carrier protein found in the mitochondrial inner membrane. Once in the matrix, (10E,12E,14E)-16-hydroxy-9-oxooctadeca-10,12,14-trienoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (10E,12E,14E)-16-hydroxy-9-oxooctadeca-10,12,14-trienoyl-CoA and L-carnitine. (10E,12E,14E)-16-hydroxy-9-oxooctadeca-10,12,14-trienoyl-CoA then enters into the mitochondrial beta-oxidation pathway to form aceytl-CoA. Acetyl-CoA can go on to enter the TCA cycle, or it can react with L-carnitine to form L-acetylcarnitine in a reaction catalyzed by Carnitine O-acetyltransferase. This reaction can occur in both directions, and L-acetylcarnitine and CoA can react to form acetyl-CoA and L-carnitine in certain circumstances. Finally, acetyl-CoA in the cytosol can be catalyzed by acetyl-CoA carboxylase 1 to form malonyl-CoA, which inhibits the action of carnitine O-palmitoyltransferase 1, thereby preventing (10E,12E,14E)-16-hydroxy-9-oxooctadeca-10,12,14-trienoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolic

SMP0124386

Pw125842 View Pathway

Acylcarnitine (5Z,8Z,10E,12E,14Z)-Icosa-5,8,10,12,14-pentaenoylcarnitine

(5Z,8Z,10E,12E,14Z)-Icosa-5,8,10,12,14-pentaenoylcarnitine is an acylcarnitine. The general role of acylcarnitines is to transport acyl-groups, organic acids and fatty acids, from the cytoplasm into the mitochondria so that they can be broken down to produce energy. As part of this process, (5Z,8Z,10E,12E,14Z)-Icosa-5,8,10,12,14-pentaenoic acid is first transported into the cell via the long-chain fatty acid transport protein 1 (FATP1). Once inside the cell it undergoes a reaction to form an acyl-CoA derivative called (5Z,8Z,10E,12E,14Z)-Icosa-5,8,10,12,14-pentaenoyl-CoA. This reaction is facilitated by the long-chain fatty-acid CoA ligase 1 protein, which adds a CoA moiety to appropriate acyl groups. Many acyl-CoA groups will then further react with other zwitterionic compounds such as carnitine (to form acylcarnitines) and amino acids (to form acyl amides). The carnitine needed to form acylcarnitines inside the cell is transported into the cell by the organic cation/carnitine transporter 2. In forming an acylcarnitine derivative, (5Z,8Z,10E,12E,14Z)-Icosa-5,8,10,12,14-pentaenoyl-CoA reacts with L-carnitine to form (5Z,8Z,10E,12E,14Z)-Icosa-5,8,10,12,14-pentaenoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (5Z,8Z,10E,12E,14Z)-Icosa-5,8,10,12,14-pentaenoylcarnitine is moved into the mitochondrial intermembrane space. Following the reaction, the newly synthesized acylcarnitine is transported into the mitochondrial matrix by a mitochondrial carnitine/acylcarnitine carrier protein found in the mitochondrial inner membrane. Once in the matrix, (5Z,8Z,10E,12E,14Z)-Icosa-5,8,10,12,14-pentaenoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (5Z,8Z,10E,12E,14Z)-Icosa-5,8,10,12,14-pentaenoyl-CoA and L-carnitine. (5Z,8Z,10E,12E,14Z)-Icosa-5,8,10,12,14-pentaenoyl-CoA then enters into the mitochondrial beta-oxidation pathway to form aceytl-CoA. Acetyl-CoA can go on to enter the TCA cycle, or it can react with L-carnitine to form L-acetylcarnitine in a reaction catalyzed by Carnitine O-acetyltransferase. This reaction can occur in both directions, and L-acetylcarnitine and CoA can react to form acetyl-CoA and L-carnitine in certain circumstances. Finally, acetyl-CoA in the cytosol can be catalyzed by acetyl-CoA carboxylase 1 to form malonyl-CoA, which inhibits the action of carnitine O-palmitoyltransferase 1, thereby preventing (5Z,8Z,10E,12E,14Z)-Icosa-5,8,10,12,14-pentaenoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolic

SMP0124417

Pw125873 View Pathway

Acylcarnitine Tricosanoylcarnitine

Tricosanoylcarnitine is an acylcarnitine. The general role of acylcarnitines is to transport acyl-groups, organic acids and fatty acids, from the cytoplasm into the mitochondria so that they can be broken down to produce energy. As part of this process, tricosanoic acid is first transported into the cell via the long-chain fatty acid transport protein 1 (FATP1). Once inside the cell it undergoes a reaction to form an acyl-CoA derivative called tricosanoyl-CoA. This reaction is facilitated by the long-chain fatty-acid CoA ligase 1 protein, which adds a CoA moiety to appropriate acyl groups. Many acyl-CoA groups will then further react with other zwitterionic compounds such as carnitine (to form acylcarnitines) and amino acids (to form acyl amides). The carnitine needed to form acylcarnitines inside the cell is transported into the cell by the organic cation/carnitine transporter 2. In forming an acylcarnitine derivative, tricosanoyl-CoA reacts with L-carnitine to form tricosanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the tricosanoylcarnitine is moved into the mitochondrial intermembrane space. Following the reaction, the newly synthesized acylcarnitine is transported into the mitochondrial matrix by a mitochondrial carnitine/acylcarnitine carrier protein found in the mitochondrial inner membrane. Once in the matrix, tricosanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form tricosanoyl-CoA and L-carnitine. Tricosanoyl-CoA then enters into the mitochondrial beta-oxidation pathway to form aceytl-CoA. Acetyl-CoA can go on to enter the TCA cycle, or it can react with L-carnitine to form L-acetylcarnitine in a reaction catalyzed by Carnitine O-acetyltransferase. This reaction can occur in both directions, and L-acetylcarnitine and CoA can react to form acetyl-CoA and L-carnitine in certain circumstances. Finally, acetyl-CoA in the cytosol can be catalyzed by acetyl-CoA carboxylase 1 to form malonyl-CoA, which inhibits the action of carnitine O-palmitoyltransferase 1, thereby preventing tricosanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolic

SMP0124391

Pw125847 View Pathway

Acylcarnitine (5Z,14Z,17Z)-Henicosa-5,14,17-trienoylcarnitine

(5Z,14Z,17Z)-Henicosa-5,14,17-trienoylcarnitine is an acylcarnitine. The general role of acylcarnitines is to transport acyl-groups, organic acids and fatty acids, from the cytoplasm into the mitochondria so that they can be broken down to produce energy. As part of this process, (5Z,14Z,17Z)-henicosa-5,14,17-trienoic acid is first transported into the cell via the long-chain fatty acid transport protein 1 (FATP1). Once inside the cell it undergoes a reaction to form an acyl-CoA derivative called (5Z,14Z,17Z)-henicosa-5,14,17-trienoyl-CoA. This reaction is facilitated by the long-chain fatty-acid CoA ligase 1 protein, which adds a CoA moiety to appropriate acyl groups. Many acyl-CoA groups will then further react with other zwitterionic compounds such as carnitine (to form acylcarnitines) and amino acids (to form acyl amides). The carnitine needed to form acylcarnitines inside the cell is transported into the cell by the organic cation/carnitine transporter 2. In forming an acylcarnitine derivative, (5Z,14Z,17Z)-henicosa-5,14,17-trienoyl-CoA reacts with L-carnitine to form (5Z,14Z,17Z)-henicosa-5,14,17-trienoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (5Z,14Z,17Z)-henicosa-5,14,17-trienoylcarnitine is moved into the mitochondrial intermembrane space. Following the reaction, the newly synthesized acylcarnitine is transported into the mitochondrial matrix by a mitochondrial carnitine/acylcarnitine carrier protein found in the mitochondrial inner membrane. Once in the matrix, (5Z,14Z,17Z)-henicosa-5,14,17-trienoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (5Z,14Z,17Z)-henicosa-5,14,17-trienoyl-CoA and L-carnitine. (5Z,14Z,17Z)-Henicosa-5,14,17-trienoyl-CoA then enters into the mitochondrial beta-oxidation pathway to form aceytl-CoA. Acetyl-CoA can go on to enter the TCA cycle, or it can react with L-carnitine to form L-acetylcarnitine in a reaction catalyzed by Carnitine O-acetyltransferase. This reaction can occur in both directions, and L-acetylcarnitine and CoA can react to form acetyl-CoA and L-carnitine in certain circumstances. Finally, acetyl-CoA in the cytosol can be catalyzed by acetyl-CoA carboxylase 1 to form malonyl-CoA, which inhibits the action of carnitine O-palmitoyltransferase 1, thereby preventing (5Z,14Z,17Z)-henicosa-5,14,17-trienoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolic
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