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PathWhiz ID Pathway Meta Data

PW125843

Pw125843 View Pathway
metabolic

Acylcarnitine (5Z,8Z,10E,14Z,17Z)-12-hydroxyicosa-5,8,10,14,17-pentaenoylcarnitine

Homo sapiens
(5Z,8Z,10E,14Z,17Z)-12-hydroxyicosa-5,8,10,14,17-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,14Z,17Z)-12-hydroxyicosa-5,8,10,14,17-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,14Z,17Z)-12-hydroxyicosa-5,8,10,14,17-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,14Z,17Z)-12-hydroxyicosa-5,8,10,14,17-pentaenoyl-CoA reacts with L-carnitine to form (5Z,8Z,10E,14Z,17Z)-12-hydroxyicosa-5,8,10,14,17-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,14Z,17Z)-12-hydroxyicosa-5,8,10,14,17-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,14Z,17Z)-12-hydroxyicosa-5,8,10,14,17-pentaenoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (5Z,8Z,10E,14Z,17Z)-12-hydroxyicosa-5,8,10,14,17-pentaenoyl-CoA and L-carnitine. (5Z,8Z,10E,14Z,17Z)-12-hydroxyicosa-5,8,10,14,17-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,14Z,17Z)-12-hydroxyicosa-5,8,10,14,17-pentaenoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

PW125839

Pw125839 View Pathway
metabolic

Acylcarnitine (5Z,8Z,10E,12S,14Z)-12-hydroxyicosa-5,8,10,14-tetraenoylcarnitine

Homo sapiens
(5Z,8Z,10E,12S,14Z)-12-hydroxyicosa-5,8,10,14-tetraenoylcarnitine 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,12S,14Z)-12-hydroxyicosa-5,8,10,14-tetraenoic 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,12S,14Z)-12-hydroxyicosa-5,8,10,14-tetraenoyl-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,12S,14Z)-12-hydroxyicosa-5,8,10,14-tetraenoyl-CoA reacts with L-carnitine to form (5Z,8Z,10E,12S,14Z)-12-hydroxyicosa-5,8,10,14-tetraenoylcarnitine. 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,12S,14Z)-12-hydroxyicosa-5,8,10,14-tetraenoylcarnitine 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,12S,14Z)-12-hydroxyicosa-5,8,10,14-tetraenoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (5Z,8Z,10E,12S,14Z)-12-hydroxyicosa-5,8,10,14-tetraenoyl-CoA and L-carnitine. (5Z,8Z,10E,12S,14Z)-12-hydroxyicosa-5,8,10,14-tetraenoyl-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,12S,14Z)-12-hydroxyicosa-5,8,10,14-tetraenoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

PW125837

Pw125837 View Pathway
metabolic

Acylcarnitine (5Z,8Z,10E,12S,14Z)-12-hydroperoxyicosa-5,8,10,14-tetraenoylcarnitine

Homo sapiens
(5Z,8Z,10E,12S,14Z)-12-hydroperoxyicosa-5,8,10,14-tetraenoylcarnitine 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,12S,14Z)-12-hydroperoxyicosa-5,8,10,14-tetraenoic 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,12S,14Z)-12-hydroperoxyicosa-5,8,10,14-tetraenoyl-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,12S,14Z)-12-hydroperoxyicosa-5,8,10,14-tetraenoyl-CoA reacts with L-carnitine to form (5Z,8Z,10E,12S,14Z)-12-hydroperoxyicosa-5,8,10,14-tetraenoylcarnitine. 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,12S,14Z)-12-hydroperoxyicosa-5,8,10,14-tetraenoylcarnitine 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,12S,14Z)-12-hydroperoxyicosa-5,8,10,14-tetraenoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (5Z,8Z,10E,12S,14Z)-12-hydroperoxyicosa-5,8,10,14-tetraenoyl-CoA and L-carnitine. (5Z,8Z,10E,12S,14Z)-12-hydroperoxyicosa-5,8,10,14-tetraenoyl-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,12S,14Z)-12-hydroperoxyicosa-5,8,10,14-tetraenoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

PW125842

Pw125842 View Pathway
metabolic

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

Homo sapiens
(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.

PW125690

Pw125690 View Pathway
metabolic

Acylcarnitine (5Z,8Z)-Pentadeca-5,8-dienoylcarnitine

Homo sapiens
(5Z,8Z)-Pentadeca-5,8-dienoylcarnitine 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)-pentadeca-5,8-dienoic 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)-pentadeca-5,8-dienoyl-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)-pentadeca-5,8-dienoyl-CoA reacts with L-carnitine to form (5Z,8Z)-pentadeca-5,8-dienoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (5Z,8Z)-pentadeca-5,8-dienoylcarnitine 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)-pentadeca-5,8-dienoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (5Z,8Z)-pentadeca-5,8-dienoyl-CoA and L-carnitine. (5Z,8Z)-Pentadeca-5,8-dienoyl-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)-pentadeca-5,8-dienoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

PW125004

Pw125004 View Pathway
metabolic

Acylcarnitine (5Z,8Z)-10-{3-[(1E,3S)-3-hydroxyoct-1-en-1-yl]oxiran-2-yl}deca-5,8-dienoylcarnitine

Homo sapiens
(5Z,8Z)-10-{3-[(1E,3S)-3-hydroxyoct-1-en-1-yl]oxiran-2-yl}deca-5,8-dienoylcarnitine 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)-10-{3-[(1E,3S)-3-hydroxyoct-1-en-1-yl]oxiran-2-yl}deca-5,8-dienoic 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)-10-{3-[(1E,3S)-3-hydroxyoct-1-en-1-yl]oxiran-2-yl}deca-5,8-dienoyl-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)-10-{3-[(1E,3S)-3-hydroxyoct-1-en-1-yl]oxiran-2-yl}deca-5,8-dienoyl-CoA reacts with L-carnitine to form (5Z,8Z)-10-{3-[(1E,3S)-3-hydroxyoct-1-en-1-yl]oxiran-2-yl}deca-5,8-dienoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (5Z,8Z)-10-{3-[(1E,3S)-3-hydroxyoct-1-en-1-yl]oxiran-2-yl}deca-5,8-dienoylcarnitine 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)-10-{3-[(1E,3S)-3-hydroxyoct-1-en-1-yl]oxiran-2-yl}deca-5,8-dienoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (5Z,8Z)-10-{3-[(1E,3S)-3-hydroxyoct-1-en-1-yl]oxiran-2-yl}deca-5,8-dienoyl-CoA and L-carnitine. (5Z,8Z)-10-{3-[(1E,3S)-3-hydroxyoct-1-en-1-yl]oxiran-2-yl}deca-5,8-dienoyl-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)-10-{3-[(1E,3S)-3-hydroxyoct-1-en-1-yl]oxiran-2-yl}deca-5,8-dienoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

PW125002

Pw125002 View Pathway
metabolic

Acylcarnitine (5Z,8Z)-10-[(2S,3R)-3-[(2Z)-oct-2-en-1-yl]oxiran-2-yl]deca-5,8-dienoylcarnitine

Homo sapiens
(5Z,8Z)-10-[(2S,3R)-3-[(2Z)-oct-2-en-1-yl]oxiran-2-yl]deca-5,8-dienoylcarnitine 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)-10-[(2S,3R)-3-[(2Z)-oct-2-en-1-yl]oxiran-2-yl]deca-5,8-dienoic 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)-10-[(2S,3R)-3-[(2Z)-oct-2-en-1-yl]oxiran-2-yl]deca-5,8-dienoyl-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)-10-[(2S,3R)-3-[(2Z)-oct-2-en-1-yl]oxiran-2-yl]deca-5,8-dienoyl-CoA reacts with L-carnitine to form (5Z,8Z)-10-[(2S,3R)-3-[(2Z)-oct-2-en-1-yl]oxiran-2-yl]deca-5,8-dienoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (5Z,8Z)-10-[(2S,3R)-3-[(2Z)-oct-2-en-1-yl]oxiran-2-yl]deca-5,8-dienoylcarnitine 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)-10-[(2S,3R)-3-[(2Z)-oct-2-en-1-yl]oxiran-2-yl]deca-5,8-dienoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (5Z,8Z)-10-[(2S,3R)-3-[(2Z)-oct-2-en-1-yl]oxiran-2-yl]deca-5,8-dienoyl-CoA and L-carnitine. (5Z,8Z)-10-[(2S,3R)-3-[(2Z)-oct-2-en-1-yl]oxiran-2-yl]deca-5,8-dienoyl-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)-10-[(2S,3R)-3-[(2Z)-oct-2-en-1-yl]oxiran-2-yl]deca-5,8-dienoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

PW125838

Pw125838 View Pathway
metabolic

Acylcarnitine (5Z,8S,9E,11Z,14Z)-8-hydroxyicosa-5,9,11,14-tetraenoylcarnitine

Homo sapiens
(5Z,8S,9E,11Z,14Z)-8-hydroxyicosa-5,9,11,14-tetraenoylcarnitine 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,8S,9E,11Z,14Z)-8-hydroxyicosa-5,9,11,14-tetraenoic 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,8S,9E,11Z,14Z)-8-hydroxyicosa-5,9,11,14-tetraenoyl-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,8S,9E,11Z,14Z)-8-hydroxyicosa-5,9,11,14-tetraenoyl-CoA reacts with L-carnitine to form (5Z,8S,9E,11Z,14Z)-8-hydroxyicosa-5,9,11,14-tetraenoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (5Z,8S,9E,11Z,14Z)-8-hydroxyicosa-5,9,11,14-tetraenoylcarnitine 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,8S,9E,11Z,14Z)-8-hydroxyicosa-5,9,11,14-tetraenoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (5Z,8S,9E,11Z,14Z)-8-hydroxyicosa-5,9,11,14-tetraenoyl-CoA and L-carnitine. (5Z,8S,9E,11Z,14Z)-8-hydroxyicosa-5,9,11,14-tetraenoyl-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,8S,9E,11Z,14Z)-8-hydroxyicosa-5,9,11,14-tetraenoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

PW125870

Pw125870 View Pathway
metabolic

Acylcarnitine (5Z,7Z,10Z,13Z,16Z,19Z)-4-hydroxydocosa-5,7,10,13,16,19-hexaenoylcarnitine

Homo sapiens
(5Z,7Z,10Z,13Z,16Z,19Z)-4-hydroxydocosa-5,7,10,13,16,19-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, (5Z,7Z,10Z,13Z,16Z,19Z)-4-hydroxydocosa-5,7,10,13,16,19-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 (5Z,7Z,10Z,13Z,16Z,19Z)-4-hydroxydocosa-5,7,10,13,16,19-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, (5Z,7Z,10Z,13Z,16Z,19Z)-4-hydroxydocosa-5,7,10,13,16,19-hexaenoyl-CoA reacts with L-carnitine to form (5Z,7Z,10Z,13Z,16Z,19Z)-4-hydroxydocosa-5,7,10,13,16,19-hexaenoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (5Z,7Z,10Z,13Z,16Z,19Z)-4-hydroxydocosa-5,7,10,13,16,19-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, (5Z,7Z,10Z,13Z,16Z,19Z)-4-hydroxydocosa-5,7,10,13,16,19-hexaenoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (5Z,7Z,10Z,13Z,16Z,19Z)-4-hydroxydocosa-5,7,10,13,16,19-hexaenoyl-CoA and L-carnitine. (5Z,7Z,10Z,13Z,16Z,19Z)-4-hydroxydocosa-5,7,10,13,16,19-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 (5Z,7Z,10Z,13Z,16Z,19Z)-4-hydroxydocosa-5,7,10,13,16,19-hexaenoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

PW125861

Pw125861 View Pathway
metabolic

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

Homo sapiens
(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.