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Showing 48711 - 48720 of 605359 pathways
SMPDB ID Pathway Name and Description Pathway Class Chemical Compounds Proteins

SMP0123558

Pw125014 View Pathway

Acylcarnitine (4S,5E)-4-hydroxy-6-[(1S,2R,5S)-5-hydroxy-3-oxo-2-[(2Z,5Z,8Z)-undeca-2,5,8-trien-1-yl]cyclopentyl]hex-5-enoylcarnitine

(4S,5E)-4-hydroxy-6-[(1S,2R,5S)-5-hydroxy-3-oxo-2-[(2Z,5Z,8Z)-undeca-2,5,8-trien-1-yl]cyclopentyl]hex-5-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, (4S,5E)-4-hydroxy-6-[(1S,2R,5S)-5-hydroxy-3-oxo-2-[(2Z,5Z,8Z)-undeca-2,5,8-trien-1-yl]cyclopentyl]hex-5-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 (4S,5E)-4-hydroxy-6-[(1S,2R,5S)-5-hydroxy-3-oxo-2-[(2Z,5Z,8Z)-undeca-2,5,8-trien-1-yl]cyclopentyl]hex-5-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, (4S,5E)-4-hydroxy-6-[(1S,2R,5S)-5-hydroxy-3-oxo-2-[(2Z,5Z,8Z)-undeca-2,5,8-trien-1-yl]cyclopentyl]hex-5-enoyl-CoA reacts with L-carnitine to form (4S,5E)-4-hydroxy-6-[(1S,2R,5S)-5-hydroxy-3-oxo-2-[(2Z,5Z,8Z)-undeca-2,5,8-trien-1-yl]cyclopentyl]hex-5-enoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (4S,5E)-4-hydroxy-6-[(1S,2R,5S)-5-hydroxy-3-oxo-2-[(2Z,5Z,8Z)-undeca-2,5,8-trien-1-yl]cyclopentyl]hex-5-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, (4S,5E)-4-hydroxy-6-[(1S,2R,5S)-5-hydroxy-3-oxo-2-[(2Z,5Z,8Z)-undeca-2,5,8-trien-1-yl]cyclopentyl]hex-5-enoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (4S,5E)-4-hydroxy-6-[(1S,2R,5S)-5-hydroxy-3-oxo-2-[(2Z,5Z,8Z)-undeca-2,5,8-trien-1-yl]cyclopentyl]hex-5-enoyl-CoA and L-carnitine. (4S,5E)-4-hydroxy-6-[(1S,2R,5S)-5-hydroxy-3-oxo-2-[(2Z,5Z,8Z)-undeca-2,5,8-trien-1-yl]cyclopentyl]hex-5-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 (4S,5E)-4-hydroxy-6-[(1S,2R,5S)-5-hydroxy-3-oxo-2-[(2Z,5Z,8Z)-undeca-2,5,8-trien-1-yl]cyclopentyl]hex-5-enoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolic

SMP0123563

Pw125019 View Pathway

Acylcarnitine 5-hydroxy-2-propylpentanoylcarnitine

5-hydroxy-2-propylpentanoylcarnitine 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, 5-hydroxy-2-propylpentanoic 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 5-hydroxy-2-propylpentanoyl-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, 5-hydroxy-2-propylpentanoyl-CoA reacts with L-carnitine to form 5-hydroxy-2-propylpentanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 5-hydroxy-2-propylpentanoylcarnitine 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, 5-hydroxy-2-propylpentanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 5-hydroxy-2-propylpentanoyl-CoA and L-carnitine. 5-hydroxy-2-propylpentanoyl-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 5-hydroxy-2-propylpentanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolic

SMP0123584

Pw125040 View Pathway

Acylcarnitine 3-Methylnonanoylcarnitine

3-Methylnonanoylcarnitine 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, 3-methylnonanoic 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 3-methylnonanoyl-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, 3-methylnonanoyl-CoA reacts with L-carnitine to form 3-methylnonanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 3-methylnonanoylcarnitine 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, 3-methylnonanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 3-methylnonanoyl-CoA and L-carnitine. 3-Methylnonanoyl-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 3-methylnonanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolic

SMP0123577

Pw125033 View Pathway

Acylcarnitine (4Z,7Z,10E,12E,16Z)-18-(3-ethylcycloprop-1-en-1-yl)-14-hydroxyoctadeca-4,7,10,12,16-pentaenoylcarnitine

(4Z,7Z,10E,12E,16Z)-18-(3-ethylcycloprop-1-en-1-yl)-14-hydroxyoctadeca-4,7,10,12,16-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,10E,12E,16Z)-18-(3-ethylcycloprop-1-en-1-yl)-14-hydroxyoctadeca-4,7,10,12,16-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,10E,12E,16Z)-18-(3-ethylcycloprop-1-en-1-yl)-14-hydroxyoctadeca-4,7,10,12,16-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,10E,12E,16Z)-18-(3-ethylcycloprop-1-en-1-yl)-14-hydroxyoctadeca-4,7,10,12,16-pentaenoyl-CoA reacts with L-carnitine to form (4Z,7Z,10E,12E,16Z)-18-(3-ethylcycloprop-1-en-1-yl)-14-hydroxyoctadeca-4,7,10,12,16-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,10E,12E,16Z)-18-(3-ethylcycloprop-1-en-1-yl)-14-hydroxyoctadeca-4,7,10,12,16-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,10E,12E,16Z)-18-(3-ethylcycloprop-1-en-1-yl)-14-hydroxyoctadeca-4,7,10,12,16-pentaenoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (4Z,7Z,10E,12E,16Z)-18-(3-ethylcycloprop-1-en-1-yl)-14-hydroxyoctadeca-4,7,10,12,16-pentaenoyl-CoA and L-carnitine. (4Z,7Z,10E,12E,16Z)-18-(3-ethylcycloprop-1-en-1-yl)-14-hydroxyoctadeca-4,7,10,12,16-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,10E,12E,16Z)-18-(3-ethylcycloprop-1-en-1-yl)-14-hydroxyoctadeca-4,7,10,12,16-pentaenoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolic

SMP0123570

Pw125026 View Pathway

Acylcarnitine 5-[(3S)-1,2-dithiolan-3-yl]pentanoylcarnitine

5-[(3S)-1,2-dithiolan-3-yl]pentanoylcarnitine 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, 5-[(3S)-1,2-dithiolan-3-yl]pentanoic 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 5-[(3S)-1,2-dithiolan-3-yl]pentanoyl-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, 5-[(3S)-1,2-dithiolan-3-yl]pentanoyl-CoA reacts with L-carnitine to form 5-[(3S)-1,2-dithiolan-3-yl]pentanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 5-[(3S)-1,2-dithiolan-3-yl]pentanoylcarnitine 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, 5-[(3S)-1,2-dithiolan-3-yl]pentanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 5-[(3S)-1,2-dithiolan-3-yl]pentanoyl-CoA and L-carnitine. 5-[(3S)-1,2-dithiolan-3-yl]pentanoyl-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 5-[(3S)-1,2-dithiolan-3-yl]pentanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolic

SMP0123565

Pw125021 View Pathway

Acylcarnitine 4-hydroxy-2-propylpentanoylcarnitine

4-hydroxy-2-propylpentanoylcarnitine 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, 4-hydroxy-2-propylpentanoic 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 4-hydroxy-2-propylpentanoyl-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, 4-hydroxy-2-propylpentanoyl-CoA reacts with L-carnitine to form 4-hydroxy-2-propylpentanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 4-hydroxy-2-propylpentanoylcarnitine 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, 4-hydroxy-2-propylpentanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 4-hydroxy-2-propylpentanoyl-CoA and L-carnitine. 4-hydroxy-2-propylpentanoyl-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 4-hydroxy-2-propylpentanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolic

SMP0123459

Pw124915 View Pathway

Acylcarnitine 4-Oxononanoylcarnitine

4-Oxononanoylcarnitine 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, 4-oxononanoic 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 4-oxononanoyl-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, 4-oxononanoyl-CoA reacts with L-carnitine to form 4-oxononanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 4-oxononanoylcarnitine 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, 4-oxononanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 4-oxononanoyl-CoA and L-carnitine. 4-Oxononanoyl-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 4-oxononanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolic

SMP0123439

Pw124895 View Pathway

Acylcarnitine 3-hydroxynona-4,7-dienoylcarnitine

3-hydroxynona-4,7-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, 3-hydroxynona-4,7-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 3-hydroxynona-4,7-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, 3-hydroxynona-4,7-dienoyl-CoA reacts with L-carnitine to form 3-hydroxynona-4,7-dienoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 3-hydroxynona-4,7-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, 3-hydroxynona-4,7-dienoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 3-hydroxynona-4,7-dienoyl-CoA and L-carnitine. 3-hydroxynona-4,7-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 3-hydroxynona-4,7-dienoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolic

SMP0123471

Pw124927 View Pathway

Acylcarnitine 13-(3-methyl-5-pentylfuran-2-yl)tridecanoylcarnitine

13-(3-methyl-5-pentylfuran-2-yl)tridecanoylcarnitine 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, 13-(3-methyl-5-pentylfuran-2-yl)tridecanoic 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 13-(3-methyl-5-pentylfuran-2-yl)tridecanoyl-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, 13-(3-methyl-5-pentylfuran-2-yl)tridecanoyl-CoA reacts with L-carnitine to form 13-(3-methyl-5-pentylfuran-2-yl)tridecanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 13-(3-methyl-5-pentylfuran-2-yl)tridecanoylcarnitine 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, 13-(3-methyl-5-pentylfuran-2-yl)tridecanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 13-(3-methyl-5-pentylfuran-2-yl)tridecanoyl-CoA and L-carnitine. 13-(3-methyl-5-pentylfuran-2-yl)tridecanoyl-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 13-(3-methyl-5-pentylfuran-2-yl)tridecanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolic

SMP0123466

Pw124922 View Pathway

Acylcarnitine 7-[(1R)-2-[(1E,3S)-3-hydroxyoct-1-en-1-yl]-5-oxocyclopent-2-en-1-yl]heptanoylcarnitine

7-[(1R)-2-[(1E,3S)-3-hydroxyoct-1-en-1-yl]-5-oxocyclopent-2-en-1-yl]heptanoylcarnitine 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, 7-[(1R)-2-[(1E,3S)-3-hydroxyoct-1-en-1-yl]-5-oxocyclopent-2-en-1-yl]heptanoic 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 7-[(1R)-2-[(1E,3S)-3-hydroxyoct-1-en-1-yl]-5-oxocyclopent-2-en-1-yl]heptanoyl-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, 7-[(1R)-2-[(1E,3S)-3-hydroxyoct-1-en-1-yl]-5-oxocyclopent-2-en-1-yl]heptanoyl-CoA reacts with L-carnitine to form 7-[(1R)-2-[(1E,3S)-3-hydroxyoct-1-en-1-yl]-5-oxocyclopent-2-en-1-yl]heptanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 7-[(1R)-2-[(1E,3S)-3-hydroxyoct-1-en-1-yl]-5-oxocyclopent-2-en-1-yl]heptanoylcarnitine 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, 7-[(1R)-2-[(1E,3S)-3-hydroxyoct-1-en-1-yl]-5-oxocyclopent-2-en-1-yl]heptanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 7-[(1R)-2-[(1E,3S)-3-hydroxyoct-1-en-1-yl]-5-oxocyclopent-2-en-1-yl]heptanoyl-CoA and L-carnitine. 7-[(1R)-2-[(1E,3S)-3-hydroxyoct-1-en-1-yl]-5-oxocyclopent-2-en-1-yl]heptanoyl-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 7-[(1R)-2-[(1E,3S)-3-hydroxyoct-1-en-1-yl]-5-oxocyclopent-2-en-1-yl]heptanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolic
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