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Showing 368141 - 368150 of 605359 pathways
PathBank ID Pathway Name and Description Pathway Class Chemical Compounds Proteins

SMP0123462

Pw124918 View Pathway

Acylcarnitine 2-Ethyl-2-hydroxybutanedioylcarnitine

Homo sapiens
2-Ethyl-2-hydroxybutanedioylcarnitine 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-Ethyl-2-hydroxybutanedioic 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-Ethyl-2-hydroxybutanedioyl-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-Ethyl-2-hydroxybutanedioyl-CoA reacts with L-carnitine to form 2-Ethyl-2-hydroxybutanedioylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 2-Ethyl-2-hydroxybutanedioylcarnitine 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-Ethyl-2-hydroxybutanedioylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 2-Ethyl-2-hydroxybutanedioyl-CoA and L-carnitine. 2-Ethyl-2-hydroxybutanedioyl-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-Ethyl-2-hydroxybutanedioylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolite
Metabolic

SMP0123464

Pw124920 View Pathway

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

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

SMP0123469

Pw124925 View Pathway

Acylcarnitine 11-(3-methyl-5-propylfuran-2-yl)undecanoylcarnitine

Homo sapiens
11-(3-methyl-5-propylfuran-2-yl)undecanoylcarnitine 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-(3-methyl-5-propylfuran-2-yl)undecanoic 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-(3-methyl-5-propylfuran-2-yl)undecanoyl-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-(3-methyl-5-propylfuran-2-yl)undecanoyl-CoA reacts with L-carnitine to form 11-(3-methyl-5-propylfuran-2-yl)undecanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 11-(3-methyl-5-propylfuran-2-yl)undecanoylcarnitine 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-(3-methyl-5-propylfuran-2-yl)undecanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 11-(3-methyl-5-propylfuran-2-yl)undecanoyl-CoA and L-carnitine. 11-(3-methyl-5-propylfuran-2-yl)undecanoyl-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-(3-methyl-5-propylfuran-2-yl)undecanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolite
Metabolic

SMP0123476

Pw124932 View Pathway

Acylcarnitine 4-ethoxy-4-oxobutanoylcarnitine

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

SMP0123481

Pw124937 View Pathway

Acylcarnitine 3-[(1S,2R,5S)-5-hydroxy-2-[(1E,3S,5Z,8Z,11Z)-3-hydroxytetradeca-1,5,8,11-tetraen-1-yl]-3-oxocyclopentyl]propanoylcarnitine

Homo sapiens
3-[(1S,2R,5S)-5-hydroxy-2-[(1E,3S,5Z,8Z,11Z)-3-hydroxytetradeca-1,5,8,11-tetraen-1-yl]-3-oxocyclopentyl]propanoylcarnitine 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-[(1S,2R,5S)-5-hydroxy-2-[(1E,3S,5Z,8Z,11Z)-3-hydroxytetradeca-1,5,8,11-tetraen-1-yl]-3-oxocyclopentyl]propanoic 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-[(1S,2R,5S)-5-hydroxy-2-[(1E,3S,5Z,8Z,11Z)-3-hydroxytetradeca-1,5,8,11-tetraen-1-yl]-3-oxocyclopentyl]propanoyl-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-[(1S,2R,5S)-5-hydroxy-2-[(1E,3S,5Z,8Z,11Z)-3-hydroxytetradeca-1,5,8,11-tetraen-1-yl]-3-oxocyclopentyl]propanoyl-CoA reacts with L-carnitine to form 3-[(1S,2R,5S)-5-hydroxy-2-[(1E,3S,5Z,8Z,11Z)-3-hydroxytetradeca-1,5,8,11-tetraen-1-yl]-3-oxocyclopentyl]propanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 3-[(1S,2R,5S)-5-hydroxy-2-[(1E,3S,5Z,8Z,11Z)-3-hydroxytetradeca-1,5,8,11-tetraen-1-yl]-3-oxocyclopentyl]propanoylcarnitine 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-[(1S,2R,5S)-5-hydroxy-2-[(1E,3S,5Z,8Z,11Z)-3-hydroxytetradeca-1,5,8,11-tetraen-1-yl]-3-oxocyclopentyl]propanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 3-[(1S,2R,5S)-5-hydroxy-2-[(1E,3S,5Z,8Z,11Z)-3-hydroxytetradeca-1,5,8,11-tetraen-1-yl]-3-oxocyclopentyl]propanoyl-CoA and L-carnitine. 3-[(1S,2R,5S)-5-hydroxy-2-[(1E,3S,5Z,8Z,11Z)-3-hydroxytetradeca-1,5,8,11-tetraen-1-yl]-3-oxocyclopentyl]propanoyl-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-[(1S,2R,5S)-5-hydroxy-2-[(1E,3S,5Z,8Z,11Z)-3-hydroxytetradeca-1,5,8,11-tetraen-1-yl]-3-oxocyclopentyl]propanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolite
Metabolic

SMP0123488

Pw124944 View Pathway

Acylcarnitine (9E)-10-nitrooctadec-9-enoylcarnitine

Homo sapiens
(9E)-10-nitrooctadec-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, (9E)-10-nitrooctadec-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 (9E)-10-nitrooctadec-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, (9E)-10-nitrooctadec-9-enoyl-CoA reacts with L-carnitine to form (9E)-10-nitrooctadec-9-enoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (9E)-10-nitrooctadec-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, (9E)-10-nitrooctadec-9-enoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (9E)-10-nitrooctadec-9-enoyl-CoA and L-carnitine. (9E)-10-nitrooctadec-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 (9E)-10-nitrooctadec-9-enoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolite
Metabolic

SMP0123493

Pw124949 View Pathway

Acylcarnitine (10E)-11-(3,4-dimethyl-5-propylfuran-2-yl)undec-10-enoylcarnitine

Homo sapiens
(10E)-11-(3,4-dimethyl-5-propylfuran-2-yl)undec-10-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, (10E)-11-(3,4-dimethyl-5-propylfuran-2-yl)undec-10-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 (10E)-11-(3,4-dimethyl-5-propylfuran-2-yl)undec-10-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, (10E)-11-(3,4-dimethyl-5-propylfuran-2-yl)undec-10-enoyl-CoA reacts with L-carnitine to form (10E)-11-(3,4-dimethyl-5-propylfuran-2-yl)undec-10-enoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (10E)-11-(3,4-dimethyl-5-propylfuran-2-yl)undec-10-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, (10E)-11-(3,4-dimethyl-5-propylfuran-2-yl)undec-10-enoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (10E)-11-(3,4-dimethyl-5-propylfuran-2-yl)undec-10-enoyl-CoA and L-carnitine. (10E)-11-(3,4-dimethyl-5-propylfuran-2-yl)undec-10-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 (10E)-11-(3,4-dimethyl-5-propylfuran-2-yl)undec-10-enoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolite
Metabolic

SMP0123495

Pw124951 View Pathway

Acylcarnitine 11-(5-ethyl-3,4-dimethylfuran-2-yl)undecanoylcarnitine

Homo sapiens
11-(5-ethyl-3,4-dimethylfuran-2-yl)undecanoylcarnitine 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-(5-ethyl-3,4-dimethylfuran-2-yl)undecanoic 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-(5-ethyl-3,4-dimethylfuran-2-yl)undecanoyl-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-(5-ethyl-3,4-dimethylfuran-2-yl)undecanoyl-CoA reacts with L-carnitine to form 11-(5-ethyl-3,4-dimethylfuran-2-yl)undecanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 11-(5-ethyl-3,4-dimethylfuran-2-yl)undecanoylcarnitine 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-(5-ethyl-3,4-dimethylfuran-2-yl)undecanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 11-(5-ethyl-3,4-dimethylfuran-2-yl)undecanoyl-CoA and L-carnitine. 11-(5-ethyl-3,4-dimethylfuran-2-yl)undecanoyl-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-(5-ethyl-3,4-dimethylfuran-2-yl)undecanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolite
Metabolic

SMP0123500

Pw124956 View Pathway

Acylcarnitine 11-(5-heptyl-3-methylfuran-2-yl)undecanoylcarnitine

Homo sapiens
11-(5-heptyl-3-methylfuran-2-yl)undecanoylcarnitine 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-(5-heptyl-3-methylfuran-2-yl)undecanoic 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-(5-heptyl-3-methylfuran-2-yl)undecanoyl-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-(5-heptyl-3-methylfuran-2-yl)undecanoyl-CoA reacts with L-carnitine to form 11-(5-heptyl-3-methylfuran-2-yl)undecanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 11-(5-heptyl-3-methylfuran-2-yl)undecanoylcarnitine 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-(5-heptyl-3-methylfuran-2-yl)undecanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 11-(5-heptyl-3-methylfuran-2-yl)undecanoyl-CoA and L-carnitine. 11-(5-heptyl-3-methylfuran-2-yl)undecanoyl-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-(5-heptyl-3-methylfuran-2-yl)undecanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolite
Metabolic

SMP0123507

Pw124963 View Pathway

Acylcarnitine 15-(3-methyl-5-pentylfuran-2-yl)pentadecanoylcarnitine

Homo sapiens
15-(3-methyl-5-pentylfuran-2-yl)pentadecanoylcarnitine 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, 15-(3-methyl-5-pentylfuran-2-yl)pentadecanoic 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 15-(3-methyl-5-pentylfuran-2-yl)pentadecanoyl-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, 15-(3-methyl-5-pentylfuran-2-yl)pentadecanoyl-CoA reacts with L-carnitine to form 15-(3-methyl-5-pentylfuran-2-yl)pentadecanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 15-(3-methyl-5-pentylfuran-2-yl)pentadecanoylcarnitine 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, 15-(3-methyl-5-pentylfuran-2-yl)pentadecanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 15-(3-methyl-5-pentylfuran-2-yl)pentadecanoyl-CoA and L-carnitine. 15-(3-methyl-5-pentylfuran-2-yl)pentadecanoyl-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 15-(3-methyl-5-pentylfuran-2-yl)pentadecanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
Metabolite
Metabolic
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